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Transitional cell carcinoma of the upper urinary tract is rare. Tumors arising in the renal pelvis and calyces should be treated with nephroureterectomy, irrespective of stage and grade. Low-grade, low-stage ureteral tumors may be treated with conservative resection in order to preserve renal function. High-grade, more advanced ureteral tumors should be treated with nephroureterectomy because of the high incidence of recurrence associated with local resection. The role of lymphadenectomy for upper tract transitional cell carcinoma has never been evaluated; however, it is recommended for better staging of the disease and for recommendations regarding adjuvant therapy. Adjuvant radiation therapy to sterilize microscopic residual disease and prevent local recurrence is logical. The role of adjuvant chemotherapy is under investigation.