A case of an unusual tumor of skeletal muscle origin is described. The tumor was located in the tunica vaginalis of the testis in a 19-year-old man. Histologic examination showed a proliferation of elongated or round cells, with clearly discernible cross striations, surrounded by abundant mature connective tissue, consistent with genital rhabdomyoma. Immunohistochemical and electron microscopic features supported this diagnosis. Rhabdomyoma must be considered in the differential diagnosis of paratesticular tumors.