Cystinuria is an autosomal recessive inherited aminoaciduria leading to nephrolithiasis. Although its prevalence is lower than other renal stone diseases, cystinuria requires an appropriate and specific support. Extracorporeal lithotripsy is relatively inefficient against cystine stones, thus limiting treatment options. The high risk of recurrence and the frequent evolution toward chronic kidney disease need stringent preventive measures based upon an abundant alkaline diuresis and sometimes therapeutic agents forming soluble mixed disulphides with cystine moieties. A close monitoring of diuresis volume, urinary pH and crystalluria prevents recurrence.