A 20 year old black female presented with a chief complaint of reduced vision in the left eye. The entrance visual acuities were 20/20 OD and light perception OS. The reduced vision OS had been present for about 20 years. The patient showed a positive Marcus-Gunn pupil OS with a slight associated anisocoria. Visual fields revealed a constricted OS field: this defect was most noticeable nasally. Intraocular pressures, as measured by applanation tonometry, were normal (OD 8; OS 20). A dilated fundus exam revealed an unremarkable OD fundus. The OS fundus, however, displayed the classic appearance of optic nerve hypoplasia. Optic nerve head hypoplasia is a rare congenital anomaly, where the optic nerve is underdeveloped and slightly smaller than in the fellow eye. The defective optic nerve has larger cup-disc ratio and a halo of hypopigmentation surrounding the disc. Retinal nerve fiber defects, such as those we found, also occur. This condition can be closely associated with other neurological defects, such as endocrine abnormalities or de Morsier's syndrome. We present dramatic photographs and a discussion of the differential diagnosis and management of this rare condition.