Following replication in additional cases and controls from Iceland and the Netherlands, we reported a genome-wide significant association of the CLDN14 locus, encoding the tight junction protein Claudin-14, on 21q22.13 with kidney stones (rs219780[C], allele frequency=79.20%, odds ratio (OR)=1.25, P=4.0 × 10−12). This evidence concerns the gene CLDN14 and nephrolithiasis.