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Objective To share our experience in percutaneous nephrolithotomy (PCNL) procedures in patients with horseshoe kidney. Materials and Methods The data of 7 patients undergoing PCNL were analyzed retrospectively. Preoperative clinical and laboratory data of patients (including complete urinalysis, complete blood count, serum biochemistry, and coagulation tests) were recorded. The stone surface area (mm2) was calculated by graph paper tracing of two dimensional projection of the stone on a plain film of the kidneys, ureters and bladder (KUB) in the anteroposterior view by investigators. In addition, per-operative and post-operative findings were evaluated. Success and complication rates (according to the classification of Clavien) were also determined. Results The mean stone surface area was 1234 (range 250-2460 mm2) mm2. Six patients were treated through a single tract, and one patient required additional access. Access was directed to the middle calyx (n=2), superior calyx (n=4), middle and inferior calyx (n=1) through the supracostal (n=2) and subcostal (n=5) areas. Mean operative time was 131 (range 70-215 minutes) minutes. Stone-free rate after single session PCNL was 71% (n=5) and increased to 86% (n=6) with a post-operative secondary ureterorenoscopy procedure. Complications including bleeding necessitating blood transfusion (Clavien grade 2) and prolonged drainage (Clavien degree 3a) were occurred in only 2 patients (24%). Conclusion PCNL is a safe and successful procedure in patients with horseshoe kidney and comparable with PNL procedures in patients with normal renal anatomy