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A true isolated superior mesenteric artery (SMA) dissection is a rare occurrence. The increasing use of diagnostic imaging studies has resulted in this rare disease being more recognized. A 68-year-old Caucasian female presented with sharp upper abdominal pain. Computed tomography (CT) of the abdomen showed dissection with thrombosis in the proximal SMA. Conservative management with bowel rest, blood pressure control, and anticoagulation relieved her symptoms. Follow-up CT showed stable dissection. Physicians should consider the diagnosis of isolated spontaneous SMA dissection after excluding more common causes. The optimal management pathway has not been firmly established. Conservative management with anticoagulation appears to be a safe first-line therapy in selected patients.