Secondary pure red cell aplasia (PRCA), as an extrahepatic manifestation of hepatitis A virus infection, has been reported on rare occasions. We report herein an unusual case of hepatitis A complicated by PRCA. In addition, we reviewed nine cases reported in the English literature. Our case of nonfulminant hepatitis A complicated by PRCA and hemolytic anemia was successfully treated with initial transfusion and corticosteroid therapy for 18 weeks. The patient’s hematologic abnormalities and liver function tests re-normalized completely. We review clinical features and effective therapeutic strategies for this disease entity.