We report the case of a male patient with a left renal pelvic stone 2.5 cm in maximum diameter and a large upper pole intrarenal arterial aneurysm on the same side. The stone was treated with percutaneous nephrolithotomy (PCNL). This procedure was feasible even in this difficult clinical setting. Puncture of a calyx located at a safe distance from the aneurysm and meticulous surgical technique were essential in realizing the best possible outcome. To our knowledge, this is the first case of PCNL performed on a patient with a renal stone and an intrarenal arterial aneurysm.