BACKGROUND

Fibrous dysplasia of the bone is a disease caused by a somatic GNAS mutation that affects craniofacial bones and can have a mass effect on different neurovascular structures. The authors present the first case of primary jugular foramen fibrous dysplasia with occlusion of the transverse and sigmoid sinuses.

OBJECTIVE

A 33-year-old man presented with a history of dizziness and occasional dysphagia over the past year. Magnetic resonance imaging showed a uniform enhanced mass in the left jugular foramen, with complete blockage of the adjacent transverse and sigmoid sinuses. The computed tomography scan revealed a cystic bone lesion of the jugular foramen. The patient underwent a gross-total removal of the tumor through an infratemporal transjugular approach with complete preservation of the lower cranial nerves.

LESSONS

This first reported case of primary jugular foramen fibrous dysplasia highlights the importance of considering this diagnosis when evaluating jugular fossa lesions. Understanding the anatomy of the infratemporal and jugular fossae, along with proficiency in microsurgical techniques, is essential for removing such tumors while preserving cranial nerve functions and the patient's quality of life. https://thejns.org/doi/10.3171/CASE24396.