Symptoms caused by squeezing ureters and abdominal vessels usually occurs after the onset of retroperitoneal fibrosis. We report a rare case of localized aortic dissection in a patient with retroperitoneal fibrosis. A 64-year-old man presented with intermitted abdominal pain was diagnosed with localized abdominal aortic dissection secondary to retroperitoneal fibrosis. After the treatment of the glucocorticoid and cyclophosphamide, both localized abdominal aortic dissection and retroperitoneal fibrosis disappeared. Imaging techniques such as ultrasound have played an important role in the diagnosis and follow-up. This is a very rare case of localized abdominal aortic dissection secondary to retroperitoneal fibrosis and then successfully cured only by the glucocorticoid and cyclophosphamide.