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Along with medulloblastomas, Atypical teratoid/rhabdoid tumors (ATRT) are embryonic in origin and histologically show rhabdoid cells and mixed portions of neuroectodermal, ectodermal, and mesenchymal cells. ATRTs are present according to the location of the tumor within the CNS. Signs of headache, vomiting, gait abnormalities, and instability are typical of cerebellar tumors. There is no clear treatment protocol for ATRTs. In this report, we present the case of a 1-year-old boy who was diagnosed with a supratentorial ATRT. The patient underwent surgery and was referred for adjuvant chemo/radiotherapy.