NUT carcinoma (nuclear protein in testis) is a rare and aggressive malignancy, primarily arising from midline structures, such as the thorax and head and neck. Brain involvement in NUT carcinoma is exceedingly rare, particularly as the initial presentation. This report presents the case of a 40-year-old male who presented with symptoms of trigeminal neuralgia and was later diagnosed with NUT carcinoma involving the brain. This case highlights the diagnostic challenges posed by this malignancy and stresses the importance of considering malignancy in patients with unexplained neurological symptoms.