Eight of nine individuals of a four-generation family had autosomal dominant central pigment epithelial and choroidal degeneration, which was not entirely consistent with previously described central retinal and choroidal degenerations. The early loss of the choriocapillaris with a relative sparing of the sensory retinal and pigment epithelial function is similar to central areolar choroidal degeneration. Ophthalmoscopically, however, the disease resembled the dominant progressive foveal degenerations; except that our patients additionally had multiple peripheral yellow-white lesions. An exceptional finding in our patients was the preservation of good visual acuity despite marked atrophy of the choriocapillaris.