A 17-year-old boy presented with a large exotropia with both eyes fixed in an abduction and upgaze, pupillary involvement since childhood. He had mild optic nerve hypoplasia in the right eye and situs inversus of the retinal vessels in the left optic disc. His ocular motility showed restriction of eye movements in all gazes. He was diagnosed with congenital fibrosis of extraocular muscles, type 2 (CFEOM2) and operated upon in a staged procedure with a satisfactory eye alignment using hang-back sutures in one eye and periosteal fixation in the other. This report highlights the surgical course and final outcome in this case of CFEOM2.