Ocular coloboma is a rare malformation which occurs as an isolated defect in healthy individuals or be part of a complex malformation syndrome of known or unknown etiology. Patients with coloboma of iris and choroid complicated with cataract should have a cataract surgery to improve their BCVA, if there is no retinal detachment.We report the case of a coloboma of iris and choroid in the right eye of a male following a consultation.The patient had a inferonasal coloboma of iris, which resulted in the pear-shaped pupil in the left eye, and opaqueness of lens, may result in the lower visual acuity. Fundus examination revealed a large inferior choroidal coloboma under the optic disc in the left eye, which was about seven-disc areas.