Joubert syndrome is a ciliopathy that has been associated with mutations in approximately 27 cilia genes and has a wide range of clinical presentations.8,11,13AHI1 was the first gene associated with Joubert syndrome,12,14 and, together with CEP290, it is one of the genes most commonly associated with retinal dystrophy and kidney disease in JBTS patients.8,11 Here, we report the generation of ahi1lri46 mutant zebrafish that exhibit retinal dystrophy and loss of distal pronephric duct cilia. This evidence concerns the gene CEP290 and inherited retinal dystrophy.