We report a case of primary, non-mucinous, non-papillary ureteral poorly differentiated adenocarcinoma. The primary focus could only be detected during surgical exploration, because computed tomography (CT), ultrasonography (US), intravenous urography (IVU) and other radiologic and endoscopic procedures revealed no primary neoplastic focus, only lymph node and various bone metastases. Total nephroureterectomy with a bladder cuff was performed to decrease pain of the patient and to remove the primary tumoral focus.