Angiomyolipomas are benign, solitary, noninvasive lesions that most often arise in the kidney. Extrarenal manifestations of these tumors include the skin, oropharynx, the abdominal wall, retroperitoneum, gastrointestinal tract, heart, lung, liver, uterus, penis, and spinal cord. We report a patient with a giant angiomyolipoma located in the anterior mediastinum. We believe this is the seventh reported case of mediastinal angiomyolipoma and the largest reported by size. It is the second reported lesion to arise in the anterior mediastinum. Distinction from other pulmonary or thoracic masses relies on the appreciation of the unique and characteristic histologic features of these mediastinal angiomyolipomas. We conclude that, although rare, angiomyolipoma should be considered in the differential diagnosis of a mediastinal tumor.