To explore challenges related to the clinical diagnosis of internal jugular venous aneurysms, and to evaluate safety and durability of their treatment methods.Six patients presenting in our Institution with internal jugular venous aneurysms from September 2007 to August 2017 were retrospectively analyzed.Internal jugular vein aneurysms were confirmed in all 6 patients. For 3 of them a surgical treatment was deemed necessary. These were 2 patients with intravenous thrombosis and 1 patient with progressive aneurysmal enlargement during the initial monitoring period. The choice of surgical technique was based on aneurysm morphology: 2 patients with saccular aneurysms underwent tangential aneurysmectomy with lateral venorrhaphy, and a patient presenting a fusiform aneurysm underwent its total excision followed by internal jugular vein ligation. Three remaining patients were managed conservatively with one of them fully regressing and the other 2 remaining asymptomatic.Internal jugular vein aneurysms are very rare and usually of benign natural history. For asymptomatic patients, conservative treatment with close follow-up is generally recommended. If any accompanying signs or symptoms are present, such as pain, swelling, evidence of thrombosis, progressive enlargement, or severe psychological stress, timely and appropriate surgical intervention should ensue.