Renal cell carcinoma (RCC) is one of the actual oncourological problems, which mirrors the growth of incidence of this disease. Horseshoe kidney is a rare disease with 2.8% incidence in population among all anatomical inborn defects. RCC of the horseshoe kidney is a rare event and occurs only in 2% of patients with horseshoe kidney. Surgical treatment is a gold standard of treatment in patients with RCC. Nevertheless, it could be challenging from the anatomical point of view in patients with horseshoe kidney. Surgical access and approach should be individualized with the proper consideration of anatomy of the kidney, tumor size, its localization and co-morbidities. Nephronsparing surgery in patients with RCC including the cases of the horseshoe kidney is the method of choice. Modern radiology, namely 3-D reconstructions of the organ, could provide an important information on all stages of preoperative investigation, as for tumor localization and relationship to normal parenchyma, peculiarities of vascular and collector systems, their relationship with the tumor. This information could allow the detailed planning of the operation volume and prevent the complications. In this article we report on the case of partial nephrectomy in a female patient with RCC.