Extrarenal extracranial malignant rhabdoid tumors (MRT) are extremely rare and typically lethal. No consensus exists on an optimal treatment approach, and, in particular, the role of radiation therapy (RT) is poorly defined. We report on three children who underwent multimodality treatment including surgery, chemotherapy (CMT), and RT. Two of these patients are alive and without evidence of disease 6½ and 7 years after diagnosis and one has expired. Our limited experience suggests that RT in the setting of CMT and surgery has the potential to prolong survival with acceptable toxicity, but outcomes are inconsistent and further study is necessary. Pediatr Blood Cancer 2008;50:167–169. © 2006 Wiley-Liss, Inc.