There have been no reports describing polypoidal choroidal vasculopathy (PCV) in eyes with retinitis pigmentosa (RP). A 63-year-old woman who had been diagnosed as having RP was referred to us because of sudden onset of blurred vision in her right eye. Funduscopic examination revealed retinal findings typical of RP in both eyes. The macular area of the right fundus showed polypoidal lesions with massive hemorrhages. Fluorescein angiography and indocyanine green angiography showed multiple polypoidal lesions. Optical coherence tomography showed a large hemorrhagic retinal pigment epithelial (RPE) detachment and polypoidal lesions. The PCV subsided after three applications of anti-vascular endothelial growth factor (VEGF) therapy and a single application of photodynamic therapy, but "mottled lesions" with hyper- and hypofluorescence appeared temporal to the macula after disappearance of hemorrhage. We present a case of PCV in an eye with RP. Further studies are necessary to clarify whether anti-VEGF therapies could affect RPE status in eyes with RP.