We encountered an infant with nevus sebaceus of Jadassohn who was born with a striking pedunculated mass on the superotemporal aspect of the anterior sclera and limbus of the left eye. In addition, two small choroidal colobomas were seen in the papillomacular bundle area of the same eye. Histopathologic examination of the excised mass revealed a mixture of ectodermal and mesodermal elements, including cartilage and bone. These findings led to the rare diagnosis of epibulbar complex choristoma.