A 13-day-old boy presented with left scrotal tumor and coronary hypospadias. Left radical orchiectomy was performed. Histological diagnosis was embryonal-type paratesticular rhabdomyosarcoma. To the authors' knowledge, this is the first reported case of paratesticular rhabdomyosarcoma in a neonate in English-language literature. Also, the association of a testicular tumor with hypospadias has not been noted.