Objectives: This study focuses on the adjacent venous structures of tumors and their treatment in patients with second cervical (C2) dumbbell-shaped peripheral nerve sheath tumors (PNSTs). Patients and methods: The authors retrospectively analyzed the clinical outcome and the venous structures involved with tumors in 16 patients with C2 dumbbell-shaped PNSTs treated surgically between 2008 and 2015. Results: The venous structures at the craniocervical junction could be visualized in all 16 patients on contrast-enhanced T1-weighted magnetic resonance imaging. The venous structures could be identified during surgery. The common locations of venous structures were noted as follows: the suboccipital cavernous sinus was located anterolateral and toward the top of the tumor; the vertebral venous plexus was located ventral to the tumor; and the vertebral artery venous plexus was shifted anteriorly and laterally by the tumor. Total excision was achieved in all 16 patients. Clinical improvements were observed in all 16 patients postoperatively. Conclusions: The C2 dumbbell-shaped PNSTs were closely related to the suboccipital venous structures. Understanding and proper management of these venous structures is critical for reduced bleeding and successful surgery. (C) 2016 The Authors. Published by Elsevier B.V.