Angiomyolipoma is a benign tumor composed of varying proportions of smooth muscle cells, blood vessels, and adipose tissue that most commonly occurs in the kidney. Sporadic lesions and lesions arising in the setting of the tuberous sclerosis complex have been reported in extrarenal sites. We present the case of an incidentally discovered angiomyolipoma in the anterior mediastinum. Thymoma was suspected clinically, and the lesion was composed mainly of spindled-to-epithelioid cells arranged in a histologic pattern reminiscent of hemangiopericytoma, a pattern that has been described in thymoma. Immunohistochemical stains revealed positivity for smooth muscle actin and HMB-45, revealing the expression of smooth muscle and melanocytic markers characteristic of angiomyolipoma and other lesions in the PEComa family. © 2008 Elsevier Inc. All rights reserved.