A 21-year-old female patient presented with gradual progressive loss of vision in both eyes of 12 years duration. Funduscopic examination revealed bone-spicule pigmentation and choroidal and retinal pigment epithelium atrophy along the vascular arcades, with macular involvement in both eyes. The patient was diagnosed with pigmented paravenous retinochoroidal atrophy which is rare disease, uncommon in females, more commonly affecting the paravascular fundus. Very few cases of macular involvement have been reported.