The disruption of the activity of multi‐protein complex, which includes atypical G‐protein coupled receptor PC1, Ca2+‐permeable channel PC2 (both mutated in ADPKD), and fibrocystin (mutated in ARPKD; Rossetti et al., 2007; Ward et al., 2002) reportedly accounts for the distorted [Ca2+]i homeostasis in cystic cells (Bergmann et al., 2018; Ghata & Cowley, 2017). Here, PKHD1 is linked to autosomal recessive polycystic kidney disease.