We herein report the rare case of a 41-year-old Japanese woman in whom a venous aneurysm in the left cephalic vein was excised under local anesthesia. Histological examination revealed significant diminution in the number and size of muscle and elastic fibers in the aneurysm wall. Conceivably, a combination of endophlebohypertrophy and a congenital focal defect of the elastic and muscle fibers might have contributed to the development of this venous aneurysm.