Introduction: Isolated superior mesenteric artery dissection (ISMAD) has severe and sudden-onset abdominal pain with little physical examination findings. Misdiagnosis of ISMAD is seen commonly in clinical practice, which may lead to severe intestinal ischemic necrosis or even death. Here, we present a patient with abdominal pain and diagnosed as ISMAD. Case Report: A 46-year-old male was admitted to the emergency department with sudden onset abdominal pain and constipation. He had hypertension and asthma in his history. There was epigastric tenderness in his physical examination. Laboratory tests revealed no pathological findings except high white blood cell count. His complaints did not alleviate despite medical treatment and contrast enhanced abdominal computed tomography was performed. The patient was diagnosed as ISMAD and underwent emergency surgery. The patient developed widespread bowel ischemic necrosis and exitus occurred. Conclusion: Emergency physicians should be aware of ISMAD and include this in differential diagnoses, especially in patients with acute onset of abdominal pain.