Introduction: Meningocele of the optic nerve is a rare condition defined as dilatation of the optic nerve by spinal fluid not due to other pathology, classically presenting with headaches or progressive visual decline and usually follows a rapidly progressive course.
Discussion: Surgical decompression is the standard treatment with improvement or arrest of progression in most cases. We describe a case of a child with multiple congenital anomalies including a left optic nerve meningocele that progressively expanded and caused displacement of the orbit laterally resulting in severe cosmetic deformity and complete blindness in the left eye. We describe our surgical decompression as well as review the literature on optic nerve meningocele.