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Background Isolated dissection of the superior mesenteric artery (IDSMA) remains a rare diagnosis. However, new diagnostic means such as computed tomography makes it possible to detect even asymptomatic patients. If patients present symptomatic on admission, the risk of bowel infarction makes immediate therapy necessary. Today, endovascular techniques are often successfully used; however, open surgery remains important for special indications. In this paper, we present two cases with IDSMA and show why open surgical repair is still important in current treatment concepts. Methods Two cases with ISDMA that presented in our department from January 1, 2014 to June 1, 2014 are described. Data collection was performed retrospectively. Additionally, a review of articles which reported small cases series on patients with IDSMA within the past five years is provided. Results Both patients underwent open surgical repair following interdisciplinary consultation. Both patients were transferred to the intensive care unit after surgical repair and needed bowel rest, nasogastric suction and intravenous fluid therapy. CT scans were performed within the first week after operation. Platelet aggregation inhibitors were used in both cases as postoperative medication. Both patients survived and are able to participate in everyday activities. Conclusion Open surgical repair remains important in cases of anatomic variants of visceral arteries and suspected bowel infarction. Therefore, it is important that knowledge about open surgical techniques still be taught and trained.