PURPOSE: The study was undertaken to evaluate the prevalence of retinitis pigmentosa (RP) in rural and urban South India. METHODS: Seven thousand seven hundred and seventy four subjects aged 40 years or more from rural and urban Tamil Nadu, underwent comprehensive ophthalmic examination out of 9576 enumerated (81.17%). After a thorough ophthalmic examination, fundus photographs were taken for documentation. Statistical analysis was done using SPSS for Windows (ver 14). RESULTS: 7461 (95.9%) subjects had fundus details seen in both eyes. Thirteen subjects (0.17%; 4 males, 9 females) were diagnosed as retinitis pigmentosa. Retinitis pigmentosa in the urban population was seen in approximately 1 in 930 persons, while 1 in 372 of rural subjects had the disorder. This figure is greater than other reports from the western populations and that of the conservative estimate of 1 in 4000. The age and gender adjusted prevalence rate of retinitis pigmentosa to national census 2001 was 0.155%. Eight subjects (61.53%) had visual acuity less than 3/60. CONCLUSION: Prevalence of RP in South India appears to be alarmingly higher in comparison to those seen in other parts of the world.