BACKGROUND: Primary congenital glaucoma is potentially blinding condition characterized by elevated intraocular pressure and optic disc cupping. It is typically bilateral and usually manifest in the first year of life. Spontaneously arrested primary congenital glaucoma can occur, but it is very rare.CASE REPORT: A 32-year-old male patient from North Shewa presented to the department of ophthalmology, Menelik II Hospital with deterioration of vision. On examination he had large corneas with horizontal diameter of 14 mm, increased axial length, faint corneal stromal opacity and Haab's striae of both eyes. Anterior chamber angles were wide open. His intraocular pressure, optic nerve head appearance and visual field in both eyes were normal. He had subluxated dense cataract of the right eye.CONCLUSION: Late presentation with sequelae of primary congenital glaucoma without optic neuropathy is possible. Regular follow-up of spontaneously arrested congenital glaucoma and scleral fixation of intraocular lens is recommended.KEYWORDS:  Megalocornea, Primary congenital glaucoma, spontaneously arrested primary congenital glaucoma, Subluxated cataract