Background: Rahbdoid tumor of kidney (RTK) is a childhood rare neoplasm, previously thought as a sarcoma variant of Wilms’ tumor, but now is recognized as a distinct pathologic entity. Method: We report a pathologically proved case of RTK, associated with brain mass in a 4.5-month-old girl that has referred for gross hematuria. Shortly after initiating chemotherapy, because of retractile vomiting, convulsion, and cerebral salt wasting syndrome. Result: A tumor mass has found in mid-brain and pons in brain CT scan. Conclusion: Because of association of RTK with same or other pathologic brain masses, CT or MR of brain is recommended for all pathologically proven rhabdoid tumors or for patients with neurologic symptoms. Keywords: Rhabdoid tumor; Kidney; BrainReferences 1. Vujanic GM. Renal tumors in early life. Current diagnostic pathology .second edition. Mosby,Philadelphia. 2006; 12:210-9. 2. Winger DI, Buyuk A, Boher S, Turi GK, Scimeca P. Radiology-pathology conference: Rhabdoid tumor of the kidney. Journal of clinical imaging. 2006; 30:132-6. 3. Chung Cj, Lorenzo R, Rayder S, Schemankewiz E, Guy CD, Cutting j, et al. Rhabdoid tumor of kidney in the children: CT finding. AJR Am J Rontgenol .1995; 23:110-5. 4. Agrons GA, Kingsman KD, Wagner BJ, Sotelo-Avila C. Rhabdoid tumor of the kidney in children: a comparative study of 21 cases. AJR Am J Rontgenol. 1997; 168:447-51. 5. Rithcey ML, Azizkhan RG, Beckwith JB. Neonatal wilms tumor. J Pediatr Surg. 1995; 30:856-9. 6. Palmer NF, Sutow W. Clinical aspects of the rhabdoid tumor of the kidney: a report of the national wilms tumor study group. Med Pediatr Oncol. 1983; 11:242-5. 7. Chung Cj, Yeh ML, Chen CC. Rhabdoid tumor of kidney with spontaneous rupture: case report and review of literature. J Pediatr Surg Int.2008; 24(4):451-3. 8. Perlman EJ. Pediatric renal tumors: practical updates for the pathologist. Pediatr Dev Pathol. 2005; 8(3):320-38. 9. Gilbert-Barness E.Potter's pathology of the fetus, infant and child.vol 2. Second edition. Mosby, Philadelphia.2007: 1351-2. 10. Sahnoun L, Jallouli M, Jouini R, Mekki M, Belgith M. Rhabdoid tumor of kidney in the children. Prog Urol. 2004; 14(1):55-8. 11. Ahmed HU, Arya M, Levvitt G, Duffy PG, Mushtaq I.Part I:Primary malignant non-Wilms'renal tumors in children. Lancet Oncol.2007; 8(8):730-7. 12. Silverberg SG, DeLellis RA, Frable WJ, LiVolsi VA, Wick MR. Silverberg's principles and practice of surgical pathology and cytopathology. Vol 2. Fourth edition. Churchill livingstone. Philadelphia. 2006: 1670-1. 13. Reinhard H, Reinert J, Beier R, Furtwängler R, Alkasser M, Rutkowski S, et al. Rhabdoid tumors in children: prognostic factors in 70 patients diagnosed in Germany. oncol rep. 2008;19(3):819-23.