In 2017, Lu et al. [3] reported four different homozygous sequence variants in DZIP1L, located at 3q22.1, and encoding the basal body protein DAZ interacting protein 1-like protein (DZIP1L) in seven children with ARPKD from four consanguineous families. The gene discussed is DZIP1L; the disease is autosomal recessive polycystic kidney disease.