The primary hyperoxalurias (PHs) are rare inherited diseases, characterized by elevated endogenous production of oxalate, and are essentially caused by defective detoxification of glyoxylate from body fluids [1]. The clinical consequences of this overproduction are due to three properties of oxalate: it is an end product of metabolism, it has a strong affinity for calcium, and the resulting salts have a very low solubility [2]. Fortunately, the body clearance of oxalate, which is quite solely accomplished by the kidney, is very efficient and, normally, plasma levels of oxalate are in the order of mol/l, with a daily production and excretion of 20 -50 mol [3][4][5].The main characteristics of oxalate turnover in man are depicted in table 1. At the physiological pH oxalic acid is virtually all dissociated and strongly binds to calcium. In aqueous solution the thermodynamic solubility product is in the order of few milligrams. Thanks to a very efficient renal elimination, plasma levels are kept well below the values corresponding to the limit of supersaturation [6]. However, this results in relatively high concentrations of oxalate in human urine, which are therefore quite always supersaturated with respect to calcium oxalate [7]. Indeed, oxalate is the main constituent of up to 70% of all renal stones [8].Most of the newly generated oxalate comes from endogenous sources, and only about 10% is absorbed in the intestine from dietary oxalate. However, the fraction of oxalate absorbed from the bowel varies greatly, and mostly depends on the presence of calcium in the diet [9]. The remaining 90% comes from endogenous sources, the principal dietary precursors being sugars, amino acids and ascorbate [10,11]. Whereas some doubt still remain about the actual contribution Schieppati A, Daina E, Sessa A, Remuzzi G (eds): Rare Kidney Diseases.