Angioleiomyoma is a solitary form of leiomyoma that usually occurs as a painful subcutaneous lesion, with a predilection for the lower extremities of middle-aged women. In this report the case of an 11-year-old boy with angioleiomyoma mimicking giant cell tumor of the tendon sheath (GCTTS) in the palm is presented. After marginal excision of the tumor, histopathologic and immunohistochemical studies confirmed a typical case of angioleiomyoma and ruled out the preoperative diagnosis of GCTTS. There was no evidence of local recurrence at the 1-year follow-up examination.