In this clinical picture, a case of atypical superonasal iris, lens and retino-choroidal coloboma has been described in a 16-year-old boy who complained of defective vision since childhood in the right eye. The right eye showed a coloboma in the supero-nasal region of the iris and lens with fundus showing a superonasal fundal coloboma sparing the disc. No separation of the intercalary membrane was noted. This case adds to a very small number of published cases with superior iris and retinochoroidal colobomas.