A 51-year-old male was admitted with a chief complaint of hematemesis. The patient had been taking mesalazine 2 g as a maintenance treatment since he was diagnosed with CD at other hospital 9 years ago. At diagnosis, the location and behavior of disease were ileal (L1) and non-stricturing, non-penetrating (B1), respectively. In 4 years after diagnosis, he visited our hospital and underwent a bowel resection surgery with small bowel-small bowel anastomosis due to a bowel perforation, and then he started azathioprine 50 mg to prevent postsurgical relapse. At that time, there was no abnormal feature in a complete blood cell count (a white blood count of 4,620/mm
3, a hemoglobin of 12.8 g/dL, and a platelet of 385,000/mm
3) and liver function test (aspartate aminotransferase [AST]/alanine aminotransferase [ALT] levels of 21/13 IU/L). Since then, however, the platelet count was gradually decreasing; 213,000/mm
3 in 6 months, 124,000/mm
3 in 12 months, 105,000/ mm3 in 18 months, and 102,000/mm
3 in 24 months. After 3 years from azathioprine initiation, we decided to reduce the azathioprine dose from 50 mg to 25 mg because we found leukopenia (3,390/mm
3) and thrombocytopenia (83,000/mm
3). In 5 years after azathioprine use, the patient visited the department of emergency presenting with hematemesis over 200 mL. The patient had a blood pressure of 126/86 mmHg, a pulse of 120 beat per minute, a temperature of 36.5°C, a respiratory rate of 20 breaths per minute, and no acute clinical presentation. The conjunctiva was not pale and jaundice was not observed in sclera. His lung and heart sounded normal. His abdominal bowel sound was normal. There was no tenderness at costovertebral angle and abdomen. Pitting edema at the lower extremity was not observed. The results of a complete blood cell count were as follows; a white blood count of 13,510/mm
3, a hemoglobin of 9.5 g/dL, and a platelet of 199,000/mm
3 (this normal platelet count might be due to hemoconcentration because it showed 76,000/mm
3 after 2 days). The results of a liver function test were within a normal range showing AST/ ALT levels of 24/20 IU/L, an alkaline phosphatase level of 50 IU/L, and a gamma glutamyl transferase of 62 IU/L. Other measurements were also not significant including a total protein level of 6.3 g/dL, an albumin level of 4.3 g/dL, and a total bilirubin level of 0.34 mg/dL. The results of an evaluation for chronic liver disease were negative for hepatitis B surface antigen, hepatitis C virus antibody, antinuclear antibody, anti-mitochondrial antibodies, anti-smooth muscle antibody, and anti-liver kidney microsomal type 1 antibody. For evaluation of hematemesis, esophagogastroduodenoscopy was performed which showed several esophageal varices (
Fig. 1A) and a focus of active bleeding from esophageal varices (
Fig. 1B). Subsequently, endoscopic variceal ligation was successfully performed for bleeding management. Splenomegaly and dilated collateral vessels around distal esophagus were found by abdominal computed tomography (CT) on admission (
Fig. 2A and
B), while the evidence of portal vein thrombosis, one of the main causes of portal hypertension, was not observed in the coronal view of CT scan (
Fig. 2C). However, these features of portal hypertension had not been found by CT scans performed 4 years ago at the time of initiation of azathioprine (
Fig. 2D). The stiffness measured with FibroScan test for liver cirrhosis was 7.6 kPa, which was below the range of liver cirrhosis. In a liver biopsy specimen, neither fibrosis nor evidence of hepatic cirrhosis, were observed (
Fig. 3). Therefore, we concluded that NCPH was potentially related to azathioprine. After intensive discussion with the patient, azathioprine treatment was withdrawn, and biologic therapy was scheduled when a relapse would occur during meticulous monitoring. A beta-blocker was added to prevent variceal bleeding due to portal hypertension. This patient has been continuing outpatient follow-up without any complications in 2 years thereafter.