Patients with ocular coloboma and its variants may exhibit notching of the iris, peripapillary staphylomas, crater-like excavation of the optic disc, funnel-shaped widening of the retrobulbar optic nerve, cup-shaped filling defects in the perioptic subarchnoid space, and concurrent anomalies of the face, skull, and brain. Combined ophthalmological and radiological evaluation identifies these lesions successfully and differentiates them from other ocular pathology.