We report a 20-year-old-Korean woman with a congenital giant pigmented nevus and angiolipoma. She was admitted our department with a large, dark, pigmented lesion on the right flank and abdomen and a slowly growing mass on the right flank area since birth. On biopsy, the specimen taken from the giant pigmented patch showed typical findings of compound nevus. The right flank mass was surgically removed. The specimen was composed of mature fat cells with an increased vascular component. These findings are compatible with angiolipoma.