Inflammatory pseudotumor is an uncommon round and spindle cell proliferative lesion of unknown etiology that occurs most commonly in the lung. But it also occurs in diverse extrapulmonary locations such as the abdomen, retroperitoneum, pelvis, heart, head and neck, upper respiratory tract, trunk, bladder and extremities. The extrapulmonary inflammatory pseudotumor is often larger, less well circumscribed and multinodular. Proximity of the tumor to vital structures or involvement of vital organs compromises the opportunity for complete resection, thus higher recurrence rates are often reported even after surgical treatment. The authors report a case of inflammatory pseudotumor originating from the common carotid artery in a 42-year-old female patient with a rapidly growing neck mass, treated by en-bloc resection of inflammatory pseudotumor and a long segment of common carotid artery followed by PTFE graft interposition.