Purpose To assess the challenges and management of carotid body tumors (CBTs). Adequate planning by the surgeon can greatly reduce adverse events of such 6 rare and difficult tumors. 7 Background 8 CBTs are rare. Despite over a century of describing surgery, controversy remains 9 surrounding details of their treatment. Most complications are considered technically preventable. Patients and methods 11 This prospective study included 13 cases diagnosed as CBT. Patients were 12 subjected to clinical evaluation, carotid duplex ultrasound, computed 13 tomography angiography scan, and sometimes MR angiography. All patients underwent CBT resection and when needed vascular reconstruction. Patients were classified into three groups depending on Shamblin classification. 15 Results 16 Mean operative time was longer in patients of Shamblin class 3 (5.4±0.77 h; range, 17 4.5–6 h; P