We report on the very rare case of a patient with a malignant melanoma in the right ureter initially hospitalized for colic pains. Ureterorenoscopy revealed a pigmented solid mass adherent to the mid-ureteral wall. Histomorphological and immunohistochemical examination of the biopsy specimen demonstrated a malignant neoplasia with HMB45, Melan A and S100 positivity establishing the diagnosis of metastatic malignant melanoma. Hence, partial ureterectomy was performed with no further evidence of disease at the time of surgical intervention. Subsequently, multiple metastases in the renal pelvis and ureter led to secondary nephroureterectomy and retroperitoneal lymphadenectomy with complete excision of the ureteral orifice. Secondary metastatic lesions of the urinary bladder wall were completely resected followed by dacarbazine-based chemotherapy and resection of retroperitoneal recurrences. Based on this case as well as on recent published reports, we propose a possible algorithm for the treatment of metastatic melanoma of the upper urinary tract.