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The pathogenesis of calcium oxalate stone formation in patients with idiopathic hypercalciuria remains incompletely understood. Several lines of evidence suggest that inherited abnormalities of mineral metabolism may contribute to stone formation, including high frequency of calcium stone disease and hypercalciuria in first degree relatives of stone formers, similar phenotypic expression of hypercalciuria of the absorptive variety in several members of an affected kindred, and breeding of male and female hypercalciuric rats increases the magnitude and frequency of hypercalciuria among offspring. The causes of hypercalciuria are reviewed and possible molecular mechanisms are discussed.