A descending aorta to vertebral venous plexus fistula is an extremely rare form of arteriovenous fistula. A 10-month-old infant was referred to the hospital for evaluation of a murmur. On examination, a continuous murmur was heard in the entire back. Transthoracic echocardiography revealed left heart chamber dilatation in the presence of preserved left ventricle systolic function. Computerized tomography angiography with 3-dimensional reconstruction, and lateral projection aortography revealed a descending aorta-vertebral venous plexus fistula measuring 4.8 mm in the aortic orifice. The fistula was embolized using an Amplatzer Vascular Plug 2.