A thirty years old Pakistani male was referred from University of Lahore, Pakistan for clinical evaluation and interpretation of his ERG. Clinical examination revealed Cone - Rod dystrophy. Systemic examination showed discolored and abnormally shaped teeth. The patient was otherwise mentally and physically normal. His ERG showed Scotopic (rod responses) within upper Tayyaba Gul Malik normal limits in right eye while markedly reduced in the left eye. Scotopic rod Associater professor and cone combined responses (indicating generalized retinal functions) were EOmphatihl.atlamyoyalobgaymL@MDyaChoo.com also decreased. Photopic (cone responses) were reduced in both eyes, with Received: December 28, 2015 more marked fall in the left eye as compared to the right eye. Accepted: March 09, 2016. Key Words: Rod-cone dystrophy, amelogenesis imperfect, Electro-retinogram, pigmentary retinopathy J is associated with amelogenesis imperfecta. Few alili Syndrome is a rare cone-rod dystrophy which cases are reported in literature so far. The very first report of Jalili syndrome was published in 1988 by an Iraqi ophthalmologist named Ismail K. Jalili. Jalili and Smith reported 29 individuals from a single highly inbred Arab family from the Gaza strip. Later, other families with this syndrome were reported mainly from middle east. Only one case from North America and Indian sub-continent are reported. We report a case of Jalili syndrome from northern areas of Pakistan. Environmental factors and genetic association of this syndrome is also mentioned with reference to the hypotheses already presented in different papers globally.