Background <p>Duplicated inferior vena cava (IVC) is a rare anatomical anomaly typically identified incidentally during imaging or surgery. While usually asymptomatic, it poses significant surgical challenges, particularly during nephrectomy. Concurrently, renal malrotation, intestinal malrotation, and May-Thurner anatomy are infrequently observed. The combination of these anomalies with papillary renal cell carcinoma has never been documented, underscoring the unique nature of this case.</p> Case presentation <p>We present the case of a 56-year-old male patient with abdominal bloating and a palpable mass. Imaging revealed a Bosniak category 4 cystic renal mass, subsequently diagnosed as papillary renal cell carcinoma, alongside duplicated IVC, malrotation of the left kidney and intestines, and May-Thurner anatomy. CT venography confirmed compression of the left IVC and iliac veins by the renal mass and calcified vessels. The patient underwent a successful radical nephrectomy with careful preoperative planning to mitigate risks associated with these anomalies. Postoperative recovery was uneventful, with complete resolution of symptoms.</p> Conclusion <p>This case illustrates a highly rare convergence of anatomical anomalies and malignancy, emphasizing the critical importance of meticulous preoperative imaging and surgical planning.</p>