Familial adenomatous polyposis (FAP) is an autosomal dominant condition caused by a mutation in APC tumor suppressor gene (chromosome 5q21). Affected patients have nearly 100 percent progression to colorectal cancer. Solitary congenital hypertrophy of retinal pigment epithelium (CHRPE) and its multi focal variant, congenital grouped pigmentation (bear tracks), are isolated ophthalmic entities and are not associated with FAP and Gardner syndrome. Patients found to have solitary or multifocal CHRPE on ophthalmoscopy can be counselled that they do not have an increased risk of colon cancer compared with that of the general population.