While it is required to characterize more patients with diverse DZIP1L mutation profiles to establish phenotypic characteristics of DZIP1L‐associated ARPKD, the available data suggest that liver involvement in DZIP1L‐associated ARPKD might be less frequent and milder than PKHD1‐associated ARPKD. Here, DZIP1L is linked to autosomal recessive polycystic kidney disease.