Yolk sac carcinoma (YST) is rare malignant tumor of germ cell origin. It most commonly arises from the gonads but extragonadal sites of origin are reported in 20% of the cases. Head and neck germ cell tumors are uncommon and only a small number are malignant. We present the case of a 48 years old man presenting with a YST of the sinonasal tract and the arterior skull base. The patient underwent an anterior craniofacial resection and postoperative radiotherapy and continues showing no evidence of disease seven years after treatment. We discuss radiological and histological features of this tumor and provide a review of three other cases of YST arising in children.