Pituitary gland duplication is a rare malformation of unknown cause that is often associated with a nasopharyngeal teratoma, among other secondary malformations. This clinical report describes a case of pituitary gland duplication with a nasopharyngeal teratoma, cleft palate, and hypothalamic hamartoma, as well as the surgical management of this patient. This case also raises the question of whether the nasopharyngeal teratoma is the cause of the pituitary duplication above and the cleft palate below or whether it is a result of the primary duplication of the notochordal process. Various theories are presented in an attempt to answer this question, but the exact cause of these malformations remains equivocal. Future research in this topic may elucidate the answer to this question.