OBJECTIVE To present a case of tubulovillous renal pelvis adenocarcinoma. METHODS/RESULTS An 81 year-old patient presented at Emergency Department with sepsis. CT scan showed a staghorn calculus with signs suggestive of pyonefrosis. A nephrectomy was performed and pathological exam revealed tubulovillous renal pelvis adenocarcinoma. CONCLUSIONS Renal pelvis adenocarcinoma is a rare disease. It is usually accompanied by chronic urinary infections, on inflammatory state and staghorn calculi. The best therapeutic option is nephrectomy and the prognosis is poor.