RESUMO
Neurofibromatosis type 1 is a congenital condition affecting neurons and connective tissue integrity including vasculature. On extremely rare occasions these patients present with venous aneurysms affecting the internal jugular vein. If they become large enough there presents a risk of rupture, thrombosis, embolization or compression of adjacent structures. In these circumstances, or when the patient becomes symptomatic, surgical exploration is warranted. We present a case of one of the largest aneurysms in the literature and one of only five associated with Neurofibromatosis type 1. A 63-year-old female who initially presented for a Hinchey III diverticulitis requiring laparotomy developed an incidentally discovered left neck swelling prior to discharge. After nonspecific clinical exam findings, imaging identified a thrombosed internal jugular vein aneurysm. Due to the risks associated with the particularly large size of our patient's aneurysm, our patient underwent surgical exploration with ligation and excision. Although several techniques have been reported, for similar presentations, we recommend this technique.