The juxtaglomerular cell tumor (JGCT) of the kidney is a rare neoplasm which commonly secretes renin. This tumor often occurs in teenagers. This paper documents the 14th adult-onset (over 30-years-old) case with a giant JGCT which measured 9.0 x 8.0 x 7.5 cm. Histologically, this tumor was composed of both vascular and tubular components. Immunohistochemically, the vascular component reacted with renin, cytokeratin 7, ulex europaeus agglutinin-1, vascular endothelial growth factor (VEGF) and Flk-1 (VEGF-R2), whereas the tubular component was positive for renin, epithelial membrane antigen (EMA), cytokeratin 7, alpha-1-antitrypsin, VEGF and Flk-1. This finding suggests that both vascular and tubular components of JGCT may promote neoplastic proliferation via an autocrine mechanism through the action of VEGF.