We report a case of cutaneous angiomyolipoma (AML) found on the anterior abdominal wall of a 3-year-old female child. Histologic examination showed a well-circumscribed nodule in the dermis composed of an intimate mixture of convoluted thick-walled blood vessels, smooth muscle, and mature fat. This lesion differs from renal AML in terms of a lack of association with tuberous sclerosis, circumscription, absence of epithelioid cells, and male predominance. We concluded that the unique features of this lesion distinguish it from other lesions such as angiomyoma, angiolipoma, myolipoma, haemangioma, and other mixed mesenchymal tumors. This case report suggests that the features considered diagnostic of AML can occur in extrarenal sites and, therefore, this diagnosis cannot be excluded on the basis of site alone.