Myelolipoma of the adrenal gland is a benign, endocrinologically inactive neoplasm composed of mature adipose tissue and a variable amount of hematopoietic elements. Rarely giant adrenal myelolipomas have been reported in literature and they are very unusual clinical entities. We describe a case in a 72 year-old woman observed at our Department of Urology for nausea, flank and abdominal pain. The surgical resected mass measured 16.5x11.5x10 cm and weighted 1 250 g. A survey of the literature on this topic is made.