Abstract
Neurofibromatosis 1 is a genetic disorder of neural crest-derived cells. Riccardi classified NF into 8 subtypes NF-I to NF-VIII. NF-V is segmental NF (Prevalence 0.0014 and 0.002%) Caused by a Postzygotic somatic mutation, Café-au-lait macules & neurofibromas in a single, unilateral segment of the body, no family history, no systemic involvement. SNF classified into true segmental & Bilateral SNF. Glaucoma is a rare manifestation ofNeurofibromatosis -1. Patients usually have cafeaulait spots, plexiform neurofibroma of upper lid, and axillary freckles. If plexiform neurofibroma is present a 50% chance of developing glaucoma exists. Glaucoma is always unilateral, usually exists at birth or shortly after birth. We present a case of bilateral congenital glaucoma in Neurofibromatosis type 1 and management.