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Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant central nervous system neoplasm predominantly found in children under the age of 3 years, and is extremely rare in adults. There is no specific clinical presentations or radiological features in reported cases of AT/RT. Diagnosis of brain AT/RT is mainly dependent on the classical pathological characteristics. We report a rare case of AT/RT arising from the trigeminal nerve and leading to progressively multiple cranial nerve palsies in a 25-year-old male patient. Microsurgical resection of the tumor has been performed and confirmed the diagnosis by postoperative pathology. To our knowledge, this is the second case of adult-onset AT/RT originating from the trigeminal nerve.