We report iridocorneal endothelial syndrome in a male who presented at the age of 16 years with a 3-year history of complaints of blurred vision, altered pupillary shape, and monocular diplopia OD. The examination was notable for unilateral effacement of the iris architecture, stretch holes, corectopia, and localized ectropion uveae. Intraocular pressures were 41 mm Hg OD and 10 mm Hg OS. Gonioscopy revealed intermittent areas of broad synechiae anterior to Schwalbe's line alternating with a clinically normal appearance. The left eye and angle were unremarkable. Specular microscopy confirmed the presence of unilateral endothelial pleomorphism and polymegathism. To our knowledge, this is the earliest reported case of iridocorneal endothelial syndrome in a young man.