Dumbbell-shaped jugular foramen schwannomas (JFS) are rare but challenging for the treatment. Surgical resection is believed to be the optimal therapy; however, postoperative dysfunction of the lower cranial nerves (CNs), tumor residual, cerebrospinal fluid (CSF) leakage, and subcutaneous hydrops are common. The current study's objectives were to describe the optimal surgical strategies for the total removal of dumbbell-shaped JFS, the functional preservation of lower CNs, and the prevention of postoperative CSF leakage.26 consecutive patients with dumbbell-shaped JFS were surgically treated between January 2014 and June 2019. All patients were operated on via two-piece lateral suboccipital approach, vascularized muscle flap was used for the repair of the dural defect after an operation. The clinical information and radiological data of these patients were retrospectively reviewed, and the optimal surgical strategies were further evaluated and discussed.The tumor was completely removed in all 26 patients, one patient developed new CN Ⅶ paralysis, and 2 developed new CN IX and Ⅹ paralysis after an operation, all patients were significantly relieved during follow up. None of them developed subcutaneous hydrops and postoperative CSF leakage. No tumor recurrence was observed during a mean follow up of 38.8 (16-69) months.Dumbbell-shaped JFS could be safely and completely removed via the two-piece lateral suboccipital approach. Postoperative CSF leakage could be effectively prevented by careful repair of the dural defect in the jugular foramen (JF) and filling the mastoid cavity with a vascularized muscular flap.Copyright © 2021 Elsevier Ltd. All rights reserved.