There is a wide variety of developmental maxillofacial anomalies secondary to defective transformation of the first pair of pharyngeal arches into adult structures. Computed tomography and magnetic resonance imaging are essential to precisely define aberrant maxillofacial anatomy and guide appropriate management. This review article provides an overview of maxillofacial embryogenesis, clinical symptoms, and characteristic imaging features of several anomalies, including choanal atresia, congenital nasal piriform aperture stenosis, dacryocystoceles, nasolacrimal duct stenosis, cleft lip and palate, micrognathia, and midline nasal masses. Finally the major features of common first pharyngeal arch syndromes are discussed.