RT Journal Article SR Electronic T1 Extrarenal retroperitoneal angiomyolipoma JF Saudi Medical Journal JO Saudi Med J FD Prince Sultan Military Medical City SP 1124 OP 1126 VO 23 IS 9 A1 Mostafa K. Mansi A1 Waleed K. Al-Khudair A1 Nasser M. Al-Bqami A1 Georg S. Wenckepach YR 2002 UL http://smj.org.sa/content/23/9/1124.abstract AB Angiomyolipoma of the retroperitoneum is extremely rare with only 7 cases reported to date in the literature. Spontaneous hemorrhage and shock is the most common presentation that requires emergency intervention. We report a case of large extrarenal retroperitoneal angiomyolipoma that illustrates a different form of presentation for such a rare tumor mimicking a large locally advanced renal parenchymal tumor. The diagnosis was made by histopathology after performing radical nephrectomy. Although rare, angiomyolipoma of the perinephric fat may have variable presentations and should be considered in the differential diagnosis of large renal tumors particularly in view of possible kidney sparing management.