Cystine uroliths form as a result of a complex metabolic disturbance in amino acid metabolism and transport. The inheritance of this disease is obscure because it does not follow a standard mendelian pattern. Uroliths are a vexing clinical problem because the recurrence rate is high. Accurate diagnosis of uroliths requires use of crystallographic methods. The renal defect leading to excessive excretion of cystine is variable. The only effective method of prevention of recurrence is use of D-penicillamine, which is poorly tolerated by some dogs.