We report a case of lipoleiomyoma which arose in retroperitoneum and presen
ted with progressively distended abdomen in a 56-yr-old woman. The tumor wa
s well encapsulated and consisted of two components, benign smooth muscle c
ells and mature adipose tissue without any atypia. It is likely to be mista
ken as extrarenal angiomyolipoma, well-differentiated liposarcoma and leiom
yoma with fatty change. We review the histologic characteristics of previou
sly reported myolipoma and describe essential points of differential diagno
sis.