We report the case of a patient with a nasopharyngeal craniopharyngioma. The presenting symptoms included a diminished visual acuity, a dysmorphic syndrome, and impaired endocrine function. The computed tomography and magnetic resonance imaging scans showed a partially cystic tumor that had destroyed most of the craniofacial sinus and anterior part of the cranial base. The patient was operated on through a rhinoseptal approach. We found 14 similar cases in the available neurosurgical and otorhinolaryngologic literature. The pathogenesis of this tumor and more especially its origin from Rathke's pouch remnants or pharyngeal hypophysis are discussed.