Malignant germ cell tumors represent 3% of neoplasms in the pediatric population. Yolk sac tumors (endodermal sinus tumors) are the most common malignant germ cell tumors in this age group. In young children, the majority of these tumors affect extragonadal sites, principally the sacrococcygeal region. Adolescents and adults usually present with a gonadal primary. Only 6% of germ cell tumors involve the extracranial head and neck. No cases have been reported involving the mandible. Here, we present the first reported case of an infant with isolated yolk sac tumor of the right mandible.