Symptomatic carotid artery atherosclerotic disease is an indication for carotid artery endarterectomy. The coexistence of carotid body tumors (CBTs) with symptomatic carotid disease is rarely encountered and adds significant challenges to vascular surgeons, with a reported overall mortality for patients who undergo endarterectomy and tumor excision of 8.8%, as opposed to the 2% for those who had CBT excision only. We describe the case of a 79-year-old female who experienced an acute cerebrovascular accident in the left hemisphere; duplex ultrasound revealed high-grade carotid stenosis in the left side and the presence of a Shamblin I CBT. The risks and benefits of the planned operation were reviewed, and the decision was made to proceed with early carotid endarterectomy and concomitant surgical resection of the tumor using the retrocarotid dissection technique. The patient recovered well, and at 11 months from the combined procedure, her neurological deficits improved significantly.