Of 31 patients with retinitis pigmentosa, 18 (58%) had lesions of the retinal pigmentary epithelium within the maculae of both eyes. Six of the 31 patients (19%) showed macular cysts or holes (five bilateral, one unilateral) without angiographic evidence of leakage from perifoveal capillaries, while seven patients (23%) had macular cysts in both eyes associated with cystoid macular edema evident by fluorescein angiography. We believe that the macular cysts or holes were caused by changes at the vitreoretinal interface, eg, vitreous traction and preretinal membranes. Other pathologic sequences may also be responsible, such as an intrinsic increase in the permeability of retinal capillaries.