Central Nervous System (CNS) embryonal tumors are a group of rare and aggressive malignancies that primarily affect the brain and spinal cord. These tumors originate from primitive or embryonic cells and typically occur in young children, although they can also affect adults. The most common types of CNS embryonal tumors include medulloblastomas, atypical teratoid/rhabdoid tumors (AT/RT), and primitive neuroectodermal tumors (PNET). These tumors exhibit rapid growth and infiltration into surrounding tissues, making them challenging to treat. Medulloblastomas, the most prevalent CNS embryonal tumors, often occur in the cerebellum and can lead to symptoms such as headaches, nausea, and problems with coordination. AT/RT, on the other hand, tends to affect very young children and presents with a combination of symptoms such as a rapidly enlarging head, vomiting, and irritability. Treatment for CNS embryonal tumors typically involves a combination of surgery, chemotherapy, and radiation therapy. Due to the aggressiveness of these tumors, a multidisciplinary approach involving neurosurgeons, oncologists, and radiation therapists is essential for optimal management. Prognosis varies depending on factors such as tumor type, location, and the age of the patient. Despite advances in treatment, the outcomes for CNS embryonal tumors can be challenging, emphasizing the need for ongoing research to develop more effective therapeutic strategies. In summary, CNS embryonal tumors are aggressive malignancies that predominantly affect the central nervous system, particularly in children. Early diagnosis and a comprehensive treatment approach are crucial in managing these tumors, but their aggressive nature poses significant challenges in achieving favorable outcomes. Ongoing research efforts aim to improve our understanding of these tumors and develop more targeted and effective treatment options.