Autosomal recessive polycystic kidney disease (ARPKD), a ciliopathy and main cause of endstage renal disease (ESRD) in children, originates from mutagenesis of the polycystic kidney and hepatic disease 1 (PKHD1) gene and loss of function of the encoded protein fibrocystin/polyductin (FPC) [4,5]. Here, PKHD1 is linked to ciliopathy.