Myelolipoma of the adrenal gland is a benign tumor comprising adipose cells and mature hematopoietic elements. Most of these lesions are small and asymptomatic. Giant myelolipomas weighing greater than 4 kg are quite rare with three other cases reported in the literature. A case is presented of a giant myelolipoma that was resected surgically. The literature is reviewed regarding this topic with emphasis on diagnostic and therapeutic implications.