This report and review of literature aimed to report a rare case of Familial Crouzon syndrome with primary optic nerve atrophy. . It is characterized by premature closure of cranial sutures, cranial deformities, midface hypoplasia, relative mandibular prognathism, hypertelorism, proptosis, strabismus and short upper lip, crowding of teeth and primary optic atrophy. We hereby report a familial case of a 16yr old girl and her father along with pedigree analysis of the trait. Our patient showed characteristic features of Crouzon syndrome and was reported very late though had signs of raised intracranial pressure with primary optic atrophy.