Recently, mutations in DAZ interacting protein 1-like (DZIP1L) have been reported in patients with ARPKD, suggesting that ARPKD is not a homogeneous disorder and DZIP1L may also be involved in its pathogenesis [4], however, whether there is a causative relationship between DZIP1L and ARPKD requires further investigation [5]. This evidence concerns the gene DZIP1L and autosomal recessive polycystic kidney disease.