Aneurysmal bone cyst (ABC) of the jugular foramen is a rare benign bone tumor of the central nervous system. Due to its deep anatomical location and adjacency to critical nerves and vessels, it is prone to preoperative misdiagnosis and poses significant surgical challenges. This article reports a 34-year-old female patient who presented with "headache and pulsatile tinnitus for more than 1 month". After multidisciplinary team (MDT) consultation, the patient underwent total microsurgical resection of the right jugular foramen lesion and skull base bone reconstruction. Postoperative pathology combined with immunohistochemistry (SATB2 positive) and molecular testing (USP6 gene rearrangement positive) confirmed the diagnosis of ABC. No residual tumor or recurrence was observed at the 4-month follow-up. This case suggests that combined multimodal imaging examinations can improve the preoperative diagnostic accuracy of jugular foramen ABC, the MDT approach can optimize treatment strategies and reduce surgical risks, and microsurgical total resection is the preferred treatment modality.

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