Abstract Background Spontaneous isolated superior mesenteric artery dissection is a rare disease that may cause bowel ischemia or aneurysm rupture and subsequent death. Thus, the establishment of a correct diagnosis in the early stage is quite important. Objective To describe the presentation of 3 patients diagnosed with spontaneous isolated supramesenteric artery dissection and briefly summarize the diagnostic procedure, treatment, and clinical course. Case Reports We experienced three cases of isolated mesenteric artery dissection in the past 5 years. A definitive diagnosis was obtained by abdominal spiral computed tomography in two cases and angiography in one case. All patients were provided anticoagulation therapy. Conclusion One patient died of bowel ischemia, 2 were discharged within 21 days without complications, and one was able to discontinue anticoagulation therapy 12 months after discharge. The remaining patient has continued warfarin, making it difficult to determine the end point of anticoagulation.