Angiomyolipoma is a benign neoplasm composed of varying admixtures of blood vessels, smooth muscle cells, and adipose tissue. Because of an increased risk of spontaneous haemorrhage, surgical approach is needed greater than 4-8 cm size. We here report our partial nephrectomy experience in the 24 cm size giant angiomyolipoma. 26-year-old woman referred to our clinic with a 24 cm size angiomyolipoma in her lower pole of right kidney. The inferior vena cava was deviated to the left by the mass. All the blood tests were normal and we offered her the choices of partial nephrectomy or nephrectomy. Right subcostal approach was used. The patient underwent resection of the mass with a safety region of 1 cm. Frozen section evaluation was consistent with angiomyolipoma and free for surgical margin. Warm ischemia time was 35 min. and intraoperative bleeding volume was 200 cc. Postoperative 2nd day the drain was taken and hospital stay was 4 days. In literature we observed very rare angiomyolipoma cases with such a large dimension treated by partial nephrectomy without arterial embolization. If technically suitable partial nephrectomy is the main chioce in this kind of benign lesions in young patients.