A 25-year-old female presented with a congenital painless growing mass on the right side of her neck with symptoms of tinnitus and difficulty breathing. Imaging revealed an aneurysm of the internal jugular vein reaching a maximum diameter of 9.2 cm, shifting the trachea and right thyroid lobe to the left side. Simple excision was sufficient to treat compression symptoms and prevent potential thrombosis and embolism. This is the first reported case of aneurysmal internal jugular vein with fibrofatty degeneration and hamartomatous wall morphology associated with compression symptoms.
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