Atypicl Teratoid/Rhabdoid Tumour is malignant embroyonal central nervous system tumour manifesting in children and composed of rhabdoid cells It may or may not be resembling a classical primitive neuroectodermal tumour, epithelial tissue and neoplastic mesenchme. A highly malignant pediatric embryonal tumor of the CNS (WHO grade IV) that exhibits rhabdoid neoplastic cells with or without non-rhabdoid components. A medulloblastoma-like and/or a primitive neuroectodermal tumor (PNET)-like component are the most common non-rhabdoid elements. A carcinomatous component or a sarcomatous component can also be present in some cases. We report two case of Atypical tertoid Rhabdoid tumour, one at the age of 3 yrs and another at the age of 9 yrs, both cases are arise from supra tentorial region.
