Introduction. Autosomal dominant polycystic kidney disease (ADPKD) described as the most common hereditary renal disease, can be associated with nephrolithiasis in 20% of cases, 5–10 times more than the general population. Approximately 3% of children with ADPKD-causing mutations have either very-early-onset or unusually rapid progressive disease, however, urolithiasis is rare in childhood. Material and methods. Anamnestic, clinical and paraclinical data were taken from the patient’s observation sheet, hospitalized in the Nephrology Department of the Mother and Child Institute. Results. An 15 years-old boy, admitted with complaints of low back pain with irradiation in the lower abdomen and periodic dysuria. Primary manifestation of about 1.5 years. The disease evolves on the background of aggravated hereditarycollateral anamnesis, with polycystic kidney disease and urolithiasis in the father and the death of the grandmother from chronic kidney disease. At the objective exam: asthenic constitutional type, periorbital edema, bilateral positive Giordani sign. Urinalysis showed erythrocyte 10-12 f/w. Serum biochemistry with no changes. The abdominal ultrasonography showed both kidneys larger than normal, and bilaterally, numerous cysts, up to 13 mm, associated with calculus on the right, 6 mm. Abdominal CT confirms the presence of multiple intraparenchymal renal cysts, some with bilateral subcapsular enlargement. Diathesis urica on the right. In the 24 hour urine examination, increased excretion of uric acid. Conclusions. Complications of ADPKD associated with urolithiasis, as well as ADPKD itself, require the exclusion of age limits with complex evaluation and monitoring of all children with kidney disease and the presence of risk factors.