Congenital germ cell tumors are uncommon. The most common site of origin is in the saccrococygeal region. Teratomas arising from the head and neck comprise a small proportion of this entity, and of these, nasopharyngeal lesions are rare. Also known by various synonyms such as hamartoma and hairy polyp, the teratoma is a well-recognized, and generally benign, clinical and histopathological entity. We present a case of a nasopharyngeal teratocarcinosarcoma associated with a cleft palate and the congenital replacement or absence of the ipsilateral Eustachian tube.