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External jugular vein aneurysm with thrombosis presenting as neck swelling is a rare clinical entity and rarely encountered in routine clinical practice. We present a case of a 45-year-old female with external jugular vein aneurysm presenting as a painful lump in the neck. Ultrasound of the neck and CT angiography showed saccular dilation of the lower end of the external jugular vein with thrombosis within the aneurysm. Saccular aneurysm of the external jugular vein is very uncommon and can lead to thrombotic complications with serious consequence.