Cystinuria is an inherited disease in which there is impaired intestinal and renal reabsorption of cystine and the dibasic amino acids arginine, lysine and ornithine, which are therefore excessively excreted in the urine. It is the most common hereditary aminoaciduria, the prevalence of which is 1:7000 worldwide. The only amino acid that crystallizes in urine is cystine, and it is the low solubility of cystine that is primarily responsible for the formation of cystine stones. The solubility of cystine depends on several different factors, namely the concentration of cystine in the urine, the pH value of the urine, the ionic strength of the urine, and the macromolecules present in the urine. Cystine stones are usually formed by the second or third decade of life. There is no difference in symptoms during the first presentation of patients with cystine stones compared to other patients with lithiasis, except that the proportion of cystine lithiasis is significantly higher in the pediatric and adolescent population compared to adults. The most common symptom of cystine stones is renal colic. It is possible to develop a urinary tract infection and kidney failure due to stone obstruction. The presence of typical transparent whitish hexagonal crystals of cystine in the urine sediment is a pathognomonic finding. It is important to analyze the composition of 24-hour urine in order to distinguish homozygotes from heterozygotes and to monitor the success of treatment. Cystine stones most often leave a ground glass opacity on a native radiograph, but they usually give a shadow with a lower density than calcium stones. In the treatment of cystine stones, two basic procedures are important, active removal of existing stones and prevention of stone recurrence. For the active removal of stones, it is necessary to apply sparing procedures, such as ESWL and endoscopic procedures. In the prophylactic treatment of cystine lithiasis, adequate hydration, alkalinization of urine, medical treatment and dietary measures are applied.