A 36-day-old male, and 2.9-kg infant who was born at 38 weeks’ gestation by uncomplicated spontaneous vaginal delivery with Apgar scores of 7 and 9 at 1 and 5 minutes was transferred to the Otolaryngology-Head & Neck Surgery of our academic, tertiary-care center for evaluation of a soft, approximately 2.5-cm long and 1.25-cm-diameter tube like process originating from the region of the left medial canthus.

There was a clear, mild mucoid discharge from an orifice at its distal end. A diagnosis of proboscis lateralis was made on the basis of the characteristic appearance of the anomaly. Associated anomalies included left nasal hypoplasia, left ocular hypoplasia, bilateral choanal atresia, and contralateral ophthalmic agenesis and blindness. Prenatal history was negative for consanguinity, exposure to alcohol, ionizing radiation, prescription medications, or toxic drugs. The patient’s parents denied any family history of blindness, craniofacial abnormalities, mental retardation, or other congenital defects. He was a first child of the family. Findings from examination of the eyes were remarkable for complete agenesis of the right eyes and microphthalmia on the left with ocular hypoplasia and no functional eye. The anterior chamber was normal.

MRI demonstrated enhancement of the proboscis central tract without evidence of intracranial communication.

PL is a rare congenital anomaly with a characteristic appearance. Computed tomography and MRI are complementary in determining the extent of the bony and soft tissue components of the anomaly. Proboscis lateralis might be associated with developmental anomalies of the ipsilateral nasal and ocular structures. Complete surgical excision at the base of the proboscis is desirable as a primary procedure if there is adequate ipsilateral nasal development or as a delayed excision if the proboscis is used in nasal reconstruction.