Our findings highlight the importance of increased serum calcium concentrations arising from attenuated CaSR signal transduction, altered vitamin D homeostasis, and increased urinary phosphate excretion via effects on FGF23, 1-α hydroxylase, and 24 hydroxylase in the pathogenesis of common idiopathic forms of KSD as well as in the rare monogenic kidney stone–associated diseases FHH, IH1, and IH2, respectively. Here, CASR is linked to familial hypocalciuric hypercalcemia.