Joubert syndrome [JS] is an autosomal  recessive neurodevelopmental disorder involving the cerebellar vermis and brainstem. Isolated Joubert syndrome is seldom noted as compared to Joubert syndrome and related disorders [JSRD]. We report a rare case of renal rickets as a complication of Joubert  syndrome and related disorders [occulo renal phenotype]. Jouberts syndrome with occulo renal defects and oro facial digital defects were diagnosed and confirmed by radiological investigations [MRI,USG,X–RAY]