Objective: Malignancy represents the second main cause of death in renal transplant patients and increase markedly by 20 years after transplantation. Method: A 41 year old male who has been on hemodialysis for 8 months for chronic renal failure, underwent living kidney transplantation from his brother. He received immunosupressive therapy of siclosporin and prednisolon. Twelve years later, due to the impairment of renal functions hemodialysis was started again and ultrasonography revealed chronic renal parenchymal disease. Graft nephrectomy was performed with the pre-diagnosis of chronic rejection. Results: Macroscopic examination revealed two well-circumscribed tumor nodules with 12 mm and 6 mm in largest diameter. Microscopically, both tumor nodules showed tubulopapillary organization. Larger tumor consisted of clear cells with TFE3 positivity, whereas the small one showed eosinophilic cells with diffuse cytokeratin7 and AMACR positivity, without any TFE3 expression. Concurrence of papillary renal cell carcinoma and renal cell carcinoma associated with Xp11.2 translocation was reported. Non-tumoral renal paranchyme revealed interstitial inflammation, tubulitis and transmural arteritis with fibrinoid necrosis compatible with type III cellular rejection. Conclusion: As far as we know this is the first case in the English literature demonstrating concurrence of papillary renal cell carcinoma and renal cell carcinoma associated with Xp11.2 translocation in a graft nephrectomy.