OBJECTIVE: An uncommon case of medullarysponge kidney with congenital hemihypertrophy complicated bynephrocalcinosis and nephrolithiasis is reported here.METHODS/RESULTS: A 29 year old female patient with multipleepisodes of renal colic is presented. Clinical features, radiologicalfindings and differential diagnosis in a patient with Cacchi-Riccidisease are discussed. At least twenty-nine cases associated withcongenital hemihypertrophy have been reported previously.CONCLUSIONS: A significant number of patients with medullarysponge kidney are asymptomatic. In many cases the diagnosis ismade when a patient is evaluated by intravenous urography for someunrelated problem.However, medullary sponge kidney has been reported inassociation with rare congenital anomalies (Beckwith-Wiedemannsyndrome and congenital hemihypertrophy) and these patients appearto be at risk of malignant neoplasms of the adrenal gland, kidney andliver, therefore they must be followed closely