Abstract
Embryonal tumors with multilayered rosettes (ETMR) are rare, highly aggressive brain neoplasms predominantly affecting children under 3 years of age. In the 2021 WHO Classification of Tumors of the Central Nervous System, ETMRs are classified as grade IV tumors, previously considered separate entities such as embryonal tumor with abundant neuropil and true rosettes (ETANTR), ependymoblastoma (EBL). We report a 13-month-old girl who presented with afebrile seizures, left hemiparesis, and status epilepticus. Neuroimaging revealed a right fronto-parieto-temporal mass with typical features of ETMR, confirmed by stereotactic biopsy. Despite supportive care and preparation for surgery, the patient's condition deteriorated rapidly, and she passed away 15 days after admission. This case highlights the typical presentation, imaging characteristics, and poor prognosis of ETMR, emphasizing the importance of early recognition and molecular testing in enhancing diagnosis and treatment strategies.