Angioleiomyoma is a benign solitary tumor that arises from the medial layer of the vein walls. We encountered a 21-year-old man who was presented with a non-tender, slowly growing, 3 cm x 5 cm-sized mass on the thenar eminence of his left hand. Histopathological examination showed the tumor to be a giant vascular leiomyoma. It is extremely rare for an angioleiomyoma to reach this size as well as occurring on the palm and this is the first report of such a case in Korea.