Objective. To report 23 patients presenting renal tumors not Wilms' tumor, of 185 patients presenting renal tumors treated on HC-INCA from 1980 to 2000, regarding to their diagnosis, treatment and outcome. Methods. It is a retrospective hospital-based study. We analyzed all the patients presenting renal tumors not Wilms' tumor, treated on HC-INCA from 1984 to July 2000. Results. There were studied 23 cases, with histologic diagnoses were: clear cell sarcoma (CCSร11 cases), renal cell carcinoma (RCCร3 cases), rabdoid tumor (RTร2 cases), renal lymphoma (RLร2 cases), mesoblastic nephoma (MNร3 cases), nefroblastomatosis (NBLร1 case), embryonary rabdomyossarcoma (REร1 case). Sixteen patients (69,5%) were submitted to unilateral nephroureterectomy; the other seven (30,5%) were submitted to incisional biopsies (4 were considered irressecable, 2 with a frozen section suggesting RL, one with a frozen section suggesting diffuse NBL). Chemotherapy was performed on 18 patients, neo-adjuvant on 4 cases, the only treatment on 3 patients (2 RL patients, 1 diffuse bilateral NBL), and adjuvant on 11 patients. Radiotherapy was used on 7 patients, always associated to chemotherapy. Regarding to outcome, 12 patients (52,2%) are alive (6 CCS, 2 RL, 2 MN, 1 RT, 1 NBL); and 4 patients (17,4%) are dead (2 CCS, 1 RCC e 1 RT). Seven patients (30,4%) lost follow-up after ยฎnishing their treatment (3 CCS, 2 RCC, 1 MN e 1 RT). Conclusions. There are a variety of tumors affecting the kidney on pediatric ages. Some of them are rare malignant neoplasms (CCS, RT); other are neoplasms more common on adulthood as RCC. Either one may found pre-malignant lesions related to Wilms' tumor (as MN or diffuse NBL) or tumors that usually to affect other sites (RL, RT). Clinical Implication: Diagnostic and therapeutic management of pediatric renal tumors not Wilms' tumor must be performed on specialized pediatric oncology centers, due to their rarity. Multidisciplinary approach provided by a experienced team offers better prognosis and outcome.