Carotid body tumour (CBT) surgery has a risk of stroke. A 57-year-old lady presented with lethargy and generalised limb pain secondary to a right parathyroid adenoma. CT scan demonstrated as an incidental finding of a left CBT. Intraoperatively the CBT was infiltrating the vessel wall. The carotid bifurcation was resected and reconstructed using a reversed saphenous vein graft. Postoperatively the patient developed right arm weakness. A CT scan showed a left hemispheric watershed infarct. On discharge (day 8 postoperatively) she had no functional deficit. Reconstruction of the internal carotid artery in patients with CBT is associated with an increased risk of stroke. This index case and literature research highlight the knowns and unknowns on stroke associated with CBT resection.