We report a case of lipoleiomyoma which arose in retroperitoneum and presented with progressively distended abdomen in a 56-yr-old woman. The tumor was well encapsulated and consisted of two components, benign smooth muscle cells and mature adipose tissue without any atypia. It is likely to be mistaken as extrarenal angiomyolipoma, well-differentiated liposarcoma and leiomyoma with fatty change. We review the histologic characteristics of previously reported myolipoma and describe essential points of differential diagnosis.