Spontaneous perinephric hemorrhage from extrarenal angiomyolipoma

We believe this is the first reported case of spontaneous perinephric hemorrhage arising from an extrarenal angiomyolipoma. To date there have been no cases reported of extrarenal angiomyolipoma associated with tuberous sclerosis. All extrarenal angiomyolipomas have been benign and treated with simple excision.