Extrarenal angiomyolipoma are benign lesions that have rarely been described in the thorax. We present the clinical, radiographic, and pathologic findings of a pulmonary angiomyolipoma in a 63-year-old woman who had no diagnosis of tuberous sclerosis or lymphangioleiomyomatosis. We believe that this report is one of the first descriptions of angiomyolipoma of the lung.