The Authors report the clinical features of cystine nephro--lithiasis in 57 patients with cystinuria observed from 1972 to 1980. The disease affects both sexes with equal frequency and severity. Symptom usually begin early, mostly between 15 and 20 years, but may develop at any age. Treatment consists of the administration of adequate oral fluids to obtain a copious urine volume, of oral alkali and of a-mercaptopropionylglycine. Successful results, from litholytic and from preventive medical treatment, were obtained in a high percentage of cases.Of the various forms of lithiasis, cystine stone formation is not one of the most common (1-2% of the cases) (10). Nevertheless conditions arising from this disorder are often complex, due to the frequency and severity of clinical manifestations which, with time, lead to irreversible impairment of renal function. Cystinuria is a complex inherited disorder characterized by excretion in the urine of large quantities of the aminoacids cystine, lysine, arginine, ornithine and of the mixed disulphide of cysteine and homocysteine. If it were not for the relative insolubility of cystine in urine,