A pure testicular rhabdomyosarcoma in infants and children is a very rare type of tumor. A 9-month-old baby boy who presented with a painless right testicular swelling underwent right inguinal radical orchiectomy with suspicion of testicular malignancy. This case was confirmed as an embryonal rhabdomyosarcoma by the histopathologic and immunohistochemical findings. We report on the case with reviews of the relevant literature.