A cutaneous angiomyolipoma, which is also known as a cutaneous angiolipoleiomyoma, is a rare benign mesenchymal tumor. Only 18 cases have been reported in the English literature. We describe a case of an angiomyolipoma presenting on the right ear helix of a 26-year-old female. The histopathologic examination revealed a typical form of an angiomyolipoma with a proliferation of mature adipocytes. As with all previously reported cases, our patient did not present with the stigmata of tuberous sclerosis. This is the 20th reported case of cutaneous angiomyolipoma and the 3rd reported case in Korea. (Ann Dermatol 21(2) 217∼220, 2009).
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