In two patients, we studied retinitis pigmentosa with preservation of the retinal pigment epithelium adjacent to and under the retinal arterioles (despite panretinal degeneration). Both patients with preserved para-arteriolar retinal pigment epithelium also exhibited a peculiarly strong hyperopia. In addition to previously reported features, these patients also had sheathing of the major vascular arcades, which suggested a vascular involvement in this uncommon form of retinitis pigmentosa.