Two brothers, 20 and 21 years of age, are reported who had a circumscribed area of atrophy of the pigment epithelium, choriocapillaris and part of the choroidal vessels in the posterior pole; they were otherwise in good health. In addition, the optic discs, particularly in the older brother had hypoplastic characteristics. Both patients were known to have had low visual acuity and nystagmus since early childhood. Electro-oculography was normal. Electroretinography and color vision tests were subnormal in both patients. Although the morphological picture showed features similar to central areolar choroidal dystrophy, familial bilateral macular colobomata, serpiginous choroiditis and some cases of cone dystrophy, there are clinical and electrophysiological reasons for differentiating the cases presented here from these other diseases.