Venous aneurysms are rare clinical entities, particularly in children, and their presentation and natural history often depend on the anatomical location and underlying etiology. We present a single case of a 12-year-old girl who presented with a palpable right supraclavicular mass. Imaging evaluation with CT, conventional venography, MRI and sonography revealed a large fusiform subclavian vein aneurysm with an unusual, mass-like fibrofatty component incorporated into the vessel wall. The girl ultimately required complete resection of the right subclavian vein with placement of a synthetic interposition graft. This case provides a radiology/pathology correlation of an entity that has not previously been described as well as an example of the utility of multiple imaging modalities to aid diagnosis and preoperative planning.