Adrenal extramedullary hematopoiesis may occur in association with various hematologic diseases. Its identification is invariably incidental, imaging of the lesion is poorly specific and the diagnosis requires the histological examination. We report a case of extramedullary hematopoiesis involving the right adrenal gland in a patient with hereditary spherocytosis. The literature on this entity has been reviewed and the differential with other conditions involving the adrenal gland and showing hematopoietic tissue infiltration is discussed.