Background and Aims: There are increasing reports on case series on spontaneous isolated mesenteric artery dissection, that is, dissections of the superior mesenteric artery and celiac artery, mainly due to improved diagnostic capacity of high-resolution computed tomography angiography performed around the clock. A few case–control studies are now available, while randomized controlled trials are awaited. Material and Methods: The present systematic review based on 97 original studies offers a comprehensive overview on risk factors, management, conservative therapy, morphological modeling of dissection, and prognosis. Results and Conclusions: Male gender, hypertension, and smoking are risk factors for isolated mesenteric artery dissection, while the frequency of diabetes mellitus is reported to be low. Large aortomesenteric angle has also been considered to be a factor for superior mesenteric artery dissection. The overwhelming majority of patients can be conservatively treated without the need of endovascular or open operations. Conservative therapy consists of blood pressure lowering therapy, analgesics, and initial bowel rest, whereas there is no support for antithrombotic agents. Complete remodeling of the dissection after conservative therapy was found in 43% at mid-term follow-up. One absolute indication for surgery and endovascular stenting of the superior mesenteric artery is development of peritonitis due to bowel infarction, which occurs in 2.1% of superior mesenteric artery dissections and none in celiac artery dissections. The most documented end-organ infarction in celiac artery dissections is splenic infarctions, which occurs in 11.2%, and is a condition that should be treated conservatively. The frequency of ruptured pseudoaneurysm in the superior mesenteric artery and celiac artery dissection is very rare, 0.4%, and none of these patients were in shock at presentation. Endovascular therapy with covered stents should be considered in these patients.