A 38-year-old woman visited our hospital complaining of decreased appetite and sensation of pressure on her abdomen. Computed tomographic scan revealed right giant renal angiomyolipoma. Partial nephrectomy was performed. The resected specimen weighed 970 grams. The histological diagnosis was consistent with angiomyolipoma. Partial nephrectomy was performed because the connection between the tumor and the kidney was in a small range and the tumor was detached easily from the surrounding tissue.