Malignancy of the sphenoid sinus is rare. Tumors may extend to adjacent anatomic structures before detection and may be easily missed. Outcomes are typically poor and vary by tumor type. Clinicians should maintain vigilance for neoplastic disease in patients presenting with headache and ocular/neurological complaints of the face/sinuses.

Malignancy of the sphenoid sinus is rare. Tumors may extend to adjacent anatomic structures before detection and may be easily missed. Outcomes are typically poor and vary by tumor type. Clinicians should maintain vigilance for neoplastic disease in patients presenting with headache and ocular/neurological complaints of the face/sinuses.