Inherited Retinal diseases are an incurable cause of visual morbidity occurring in children and young adults. Retinitis pigmentosa is one of the commonest examples of inherited retinal diseases with a prevalence of 1 per 4000 individuals [1,2]. Colobomas are known to occur in approximately 1 per 10000 people. Colobomas occur due to defective closure of the embryonic fissure between 6th – 7th week of development fetal life, and are under diagnosed as they don’t cause symptoms until they involve the disc or macula or both [3]. Retinitis pigmentosa and choroidal coloboma are known to usually exist as two separate entities. The fundus photo shows features of Retinitis pigmentosa, with an isolated chorio-retinal coloboma inferior to disc, in a 30 years old lady, who, presented with decreased peripheral vision and nyctalopia. There was no history of consanguinity. On evaluation she was found to have 6/6 vision bilaterally. Anterior segment was unremarkable in both eyes. Right eye fundus showed features of Retinitis pigmentosa. Humphrey Visual Fields 24-2 tests showed bilateral visual field defects, explaining the loss in peripheral vision. After extensive literature review, such association of unilateral coloboma in a patient with bilateral Retinitis pigmentosa, with no other systemic anomalies is extremely rare, and has been reported only in a few cases [4,5].