Extrarenal angiomyolipomas are rare lesions. An angiomyolipoma located on the right ear lobe in a 49-year-old man is reported. Pathologic examination showed a well-circumscribed subcutaneous nodule, 2 cm in diameter. The components of the tumor were confirmed by immunohistochemistry. Reactivity for HMB-45 was negative. A review of the twelve published cases, including the present, reveals that the patients age ranged from 33 to 77 years (mean 53.6), the male/female ratio was 11.1. The tumors were solitary, asymptomatic, noninvasive, located most commonly in acral skin or on the ear. The clinical impression is that of a cyst, a lipoma or a vascular tumor. Signs of tuberous sclerosis were absent in all cases. In contrast to the renal form, the cutaneous angiomyolipoma is a tumor differing in sex predominance, clinical associations, circumscription, solitariness, and HMB-45 immunoreactivity. Distinction from other mesenchymal lesions depends on recognition of traditional histologic criteria.