Extragonadal germ cell tumors are extremely rare and account for only 3%-5% of all germ cell tumors. These tumors are rarely associated with metachronous primary testicular germ cell tumors. We report the fourth case of a primary germ cell tumor occurring after the treatment of a primary CNS germ cell tumor in a 27 year-old male with embryonal cell carcinoma of the testicle 9 years after the treatment of a germ cell tumor of the pineal gland. This represents the first case of a non-seminomatous germ cell tumor of the testicle after a CNS germ cell tumor. This case illustrates the importance of long term follow-up and self-examination in patients with extragonadal germ cell tumors.