Angiomyolipoma is a tumor commonly occurring in the kidney, but occasi
onally found in extrarenal sites. Retroperitoneal angiomyolipoma with
unusual features presenting in a 39 year old woman with hypertension i
s reported in this paper. Tumor fat was inconspicuous, and present lar
gely as hibernoma-like microvesicular lipid. Tumor cells also demonstr
ated positivity for HMB-45 and S-100 protein, and by electron microsco
py showed occasional cytoplasmic striated granules indistinguishable f
rom stage II premelanosomes. However, electron microscopy and immunocy
tochemistry also confirmed the presence of a substantial myogenous com
ponent in the tumor, establishing the diagnosis of angiomyolipoma. The
implications of these findings, and the role of immunocytochemistry a
nd electron microscopy in the diagnosis of this tumor are discussed.