Oguchi disease, first described in 1907, is a rare autosomal recessive disorder characterized by congenital stationary night blindness with a unique morphological and functional abnormality of the retina. The cause, though largely unknown, is associated with ARRESTIN and RHODOPSIN KINASE gene defects. This is a case report of a 20 yr old male patient of Oguchi disease who presented with classical symptoms and morphological features in the form of Mizuo phenomenon.