Pur po se: Ocular coloboma is a rare malformation resulting from defective closure of the embryonic optic fissure. It can affect iris, retina, choroid, optic disc or ciliary body. This study reviews the clinical diagnosis and the accompanying ocular and systemic abnormalities in cases of iris coloboma. Ma te ri al and Met hod: Sixteen eyes of ten patients referred to our clinic and diagnosed as iris coloboma were included in the study and were reviewed retrospectively. Re sults: Seven cases were sporadic, and three cases were familial. Isolated iris coloboma was present in only one case, and chorioretinal involvement was present in all the others. Seven cases had involvement of the optic disc. Other ocular anomalies were unilateral mi...