A 78-year-old woman consulted the gastroenterology outpatient clinic for painless hematochezia. She had no gross hematuria or abdominal pain. Her familial and past medical histories were unremarkable. Except for the presence of red stained stool on the digital rectal examination, the physical examination and laboratory investigations were within normal limits. A colonoscopy showed a 1-cm rectal polyp that was completely removed with a snare. Macroscopic examination of the specimen revealed an ulcerated rectal polypoid lesion with granulation tissue on its surface. Histologic examination exhibited proliferation of tumor cells disposed in layers and pseudo-glandular structures. The lesion was highly vascularized. The tumor cells had central nuclei, distinct borders and a clear cytoplasm suggesting clear cell carcinoma. Immunohistochemical studies of the tumor were positive for pankeratin, CD10, EMA and RCC-ma, confirming metastatic ccRCC. An abdominal computed tomography (CT) revealed a 9-cm heterogeneous and enhancing left renal mass with a 3-cm renal vein thrombosis. A chest CT was within normal limits. The patient then underwent a left radical laparoscopic nephrectomy, leading to the diagnosis of a clear-cell RCC. The tumor measured 8 cm and was characterized as Fuhrman Grade III/IV and associated with a renal vein thrombus of 1.5 cm. The renal vein margin, the peri-renal fat and the left adrenal gland were not invaded and therefore the final pathological stage was pT3aR0. A pulmonary embolism complicated the postoperative period and the patient was started on anticoagulotherapy. The patient was considered cancer-free and no systemic adjuvant treatment was given. Chest and abdominal CT showed no signs of recurrence at 3 and 6 months. Seven months after the surgery, she presented with weakness, loss of appetite and sporadic vaginal spotting. Vaginal examination revealed a 3 and a 6-mm fragile polypoid lesion, both originating from anterior vaginal wall. There was no evidence of adenopathy or involvement of the vulva or cervix. Vaginal cytology was negative. A cystoscopy was performed to rule out a urethral diverticulum and was negative. A biopsy of both polyps was performed. Pathological analysis exhibited mucosal fragments containing foci of clear cell tumor, showing identical immunohistochemical staining features with primary tumor, such as diffuse staining with pankeratin, CD10 and RCC-ma. No systemic treatments were considered for the patient given her poor performance status. Five months following the initial episode of vaginal bleeding, the patient was admitted to the hospital for persistent heavy bleeding. Physical examination revealed an important increase in size of the vaginal metastases. Laboratory tests were unremarkable, except for a hemoglobin level of 105 g/l. The patient received external beam radiotherapy with 20 Gray in 5 fractions directed to the vaginal lesions. Bleeding then diminished substantially. However, the patient expired 6 months later because of a rapid progression of her metastatic ccRCC.