Background Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive tumors, usually affecting patients younger than 3 years of age, that are characterized by a poor prognosis. Nowadays multimodal management, including surgery, chemotherapy and radiation therapy (RT), is advocated depending on the patients’ age and tumor stage, even if no consensus exists regarding the best treatment modality. Local RT seems to be the most effective treatment in prolonging progression-free and overall survival rates, although RT might not be used on younger children because of the risk of neurocognitive and endocrine sequelae. Stereotactic radiosurgery (SRS) is a valuable alternative therapeutic option to conventional RT because of the more conformal dose delivery. The aim of this study was to review the available literature on SRS in the management of ATRT. Methods The authors carried out a systematic review of PubMed, Web of Science, and Google Scholar for clinical reports dealing with SRS for the management of ATRT. Results Nine studies describing 12 patients treated with SRS for ATRT were included in the analysis. Patient's clinical features, radiosurgical treatment characteristics, and follow-up data of the pertinent literature were reviewed critically. SRS represents a feasible and effective therapeutic option in the management of ATRT. Local control has been reported in 66.7% of cases; however, 33.3% of patients experienced poor survival because of craniospinal tumor dissemination. Conclusion SRS should be considered in the multimodal treatment of ATRT, and future studies should focus on a better definition of the role played by SRS in their management. © 2017 Elsevier Inc.