Iridoschisis is a rare condition defined as a separation of the anterior iris stroma from the posterior stroma and muscle layers. In this paper, we review current data about the epidemiology, pathophysiology, clinical characteristics and differential diagnoses of this condition and discuss the specificity of surgical treatment of concomitant ocular diseases in iridoschisis patients. Iridoschisis may pose a challenge for both an ophthalmologist in an outpatient setting and an ophthalmic surgeon. Glaucoma, primarily angle-closure glaucoma, is the most often described condition concomitant to iridoschisis. Other ocular abnormalities found relatively often in iridoschisis patients include cataract, lens subluxation and corneal abnormalities. Special attention has been paid to potential complications of cataract surgery and prevention thereof. Beside addressing the practical aspects, we point to discrepancies and suggest topics for further investigation.