Extramedullary hematopoiesis is a common complication of ineffective erythropoiesis and bone marrow replacement disorders. Because of its nonspecific presentation and radiological appearance, diagnosing focal intrahepatic extramedullary hematopoiesis is challenging and often misdiagnosed as a hepatic tumor. Herein, we describe the case of a 48-year-old male with thalassemia and AE Bart’s disease with secondary hemochromatosis and cirrhosis who developed focal intrahepatic extramedullary hematopoiesis mimicking hepatocellular carcinoma. After hepatic resection, extramedullary hematopoiesis was not observed at any site, including in the remaining liver, at the 4-year follow-up.