BACKGROUND Retinitis pigmentosa (RP) is one of the most common inherited retinal diseases, with a prevalence of about 1 in 3500 to 4500. Retinitis pigmentosa inversa is a rare variant of this disorder characterized by areas of choroidal degeneration with pigment migration and bony spicule formation in the macular area. In contrast to more typical forms of RP, this anomaly destroys central vision, leaving peripheral vision intact. METHODS A 47-year-old white male was followed for about 7 years with evidence of progressive retinal pigment epithelial atrophy and hyperpigmentation affecting both maculae. Since 1970, he had noted difficulty seeing at night as well as an acquired hearing deficit that appeared to be getting worse, ultimately impairing his ability to safely drive a truck. Medical history was positive for either chloroquine or hydroxychloroquine use for 2 to 3 years as malaria prophylaxis while he served in Vietnam. In addition, his father in Louisiana had visual loss of unknown cause. During the 7-year period, the condition progressed rapidly. The patient became virtually blind secondary to visual acuity loss with dense central and paracentral scotomas. The peripheral visual fields remained intact. After several years of extensive examinations, including laboratory, electroretinography, and genetic testing, a definitive diagnosis of RP inversa was made. CONCLUSIONS RP inversa is a rare form of tapetoretinal degeneration that is characterized by decreased central vision with normal peripheral vision. A recessive form of inheritance has been postulated but never substantiated. Although there is currently no treatment, recent studies have indicated that 15,000 IU of vitamin A palmitate daily may slow the progression of retinitis pigmentosa; however, it is unknown whether this treatment would be effective for the inverse form of RP. Differential diagnoses include Leber's congenital amaurosis, central gyrate atrophy, central areolar choroidal sclerosis, progressive cone-rod dystrophy, syphilitic retinopathy, retinal toxicity from phenothiazine use, and chloroquine/hydroxychloroquine retinopathy.