An otherwise healthy 1-month-old female infant presented with a congenital nasal appendage in the left medial canthal region with associated left-sided nasal hypoplasia. The diagnosis of proboscis lateralis was made, and a 2-stage excision and reconstruction was performed in conjunction with probing and irrigation of the lacrimal system. The patient had markedly abnormal nasal anatomy, but a patent lower canalicular system with anomalous entry in the nose and a distally stenotic upper canalicular system on the affected side. A case report and review of proboscis lateralis is provided, as treatment of this rare congenital malformation should be multidisciplinary and necessitates an informed oculoplastic surgeon or ophthalmologist's assistance.