Introduction. Percutaneous nephrolithotomy (PCNL) can be used to treat horseshoe kidney stones. However, the PCNL approach can be considered difficult due to the anatomical peculiarities of the horseshoe kidney, such as the specific calyceal shape and its malformed position. Objective. To study the possibility of using PCNL in horseshoe kidney stones and its results. Material and methods. 57-year-old patient diagnosed with congenital malformation-horseshoe kidney confirmed by CT of the urinary system organs with dynamic contrast Sol. Ultravist 370, urographic phase 15’, with irradiation dose-2251 mGy/ cm2.Tomographic sections show kidneys: right10.8cm (longitudinal), left-11.3cm (longitudinal); homogeneous parenchyma, corticomedullary layers intact, with parenchymal fusion at the lower pole with a diameter of 1.2cm, on the left calcareous inclusion distributed in the middle, lower calyces and pelvis, size 5.5x3.85cm, density +1319UH, left renal pelvis 5.1x2.6cm. Paraclinical examination reveals a massive left coralliform kidney stone. Treatment: PCNL of the left horseshoe kidney. Results. The state of «stone free» was obtained through the PCNL approach after one session, the result being confirmed by non-contrast CT: the corticomedullary layers are not altered, the renal pelvis is not dilated, without stones, solid or cystic volume formations. Conclusion. PCNL is a minimally invasive treatment technique in renal lithiasis on the malformed kidney. This approach may have limitations depending on the size of the stone and the conformational changes of the kidney. These particularities can make the intervention more difficult and increase the risk of complications, but the procedure can still be considered an effective treatment option in kidney stones on the horseshoe kidney.