Dubin-Johnson syndrome (DJS) is a benign autosomal recessive liver disorder characterised by an intermittent jaundice caused by chronic and conjugated hyperbilirubinaemia. Many mutations in multidrug resistance associated protein (MRP-2) gene have been identified in patients with DJS. Although the disease is usually asymptomatic, some patients may experience vague abdominal pain. In this report, we present a patient with steatohepatitis diagnosed as DJS through a liver biopsy. The patient, 38 years of age, male was admitted to our clinic with vague abdominal pain and jaundice. High levels of serum transaminase, triglyceride, cholesterol and bilirubin were found. Ultrasonography showed Grade II hepatosteatosis in the liver. When steatohepatitis and intracytoplasmic dPAS positive pigment which is more prominent in the perivenular region (zone 3) was observed in the liver biopsy, the patient was diagnosed as DJS coexisting with steatohepatitis.