Orbital NUT carcinoma with intracranial extension.

NUT carcinoma is a rare and aggressive malignancy driven by chromosomal rearrangements of the NUTM1 gene. Although classically arising in midline structures, involvement of the orbit is exceedingly uncommon and may mimic infectious or inflammatory diseases. We present a case of orbital NUT carcinoma with intracranial extension initially treated as an epidural empyema and orbital abscess. The atypical location and clinical presentation underscore the challenges in identification this rare malignancy. Imaging, operative findings, and pathologic evaluation ultimately established the diagnosis. This case highlights the diagnostic challenges and aggressive behavior of orbital NUT carcinoma and highlights the need for early recognition and multidisciplinary management.