We describe a 31-year-old man. Although the plasma renin activity (PRA) and plasma aldosterone concentration (PAC) were elevated, computed tomography and rapid sequence pyelography disclosed no abnormality. However, based on the findings of the small tumor (8 x 8 mm) in the kidney visualized by magnetic resonance imaging (MRI) and excessive PRA in the right renal vein, tumor resection was performed. The positive immunohistochemical staining of renin and the visualization of renin mRNA by in situ hybridization provided evidence supporting the diagnosis of juxtaglomerular cell tumor. Blood pressure, PRA, and PAC were normalized after the surgery. The observations suggest that MRI is a powerful diagnostic procedure in small juxtaglomerular cell tumor.