Abstract. Primary adenocarcinoma is a rare type of urological neoplasm. The present study reports the case of a 55-year-old man with multifocal adenocarcinoma of the renal pelvis, ureter and urinary bladder that occurred in association with a large cystic calculus and perinephric abscess. The patient had suffered from gross hematuria for 2 years and right flank pain for 2 months. Following a series of investigations, a large cystic calculus with multiple tumors in the renal pelvis and ureter was identified. Multifocal tumors and a large calculus were located in the bladder using a cystoscope. The pathological report of 3 individual biopsies revealed a moderately differentiated tubular adenocarcinoma. Right nephrectomy, ureterectomy, radical cystectomy and left ureterocutaneostomy were performed. The pathological investigation revealed a moderately differentiated adenocarcinoma of the renal pelvis, ureter and urinary bladder. No additional treatment was administered and the patient remains alive at follow-up without disease recurrence or metastasis. Although uncommon, the development of a tumor is possible in patients that possess long-standing urolithiasis, particularly when accompanied by hydronephrosis or infection.