Kohlmeier-Degos' disease (KDD) is more common in white boys and men with age of onset ranging between 3 weeks of age to 67 years old, most commonly occurring in the second and third decades. The etiology and pathogenesis, although still unknown, is speculated to be immunologic dysfunction, coagulation or fibrinolysis abnormalities, or possible viral infections. Any organ system may be involved; the clinical manifestations are a result of multifocal infarctions. Laparoscopic examination, however, shows lesions similar in appearance to the skin lesions that may involve multiple areas of the small intestine. These lesions are secondary to infarction and have also been found in the esophagus, duodenum, stomach, colon, and rectum. Ischemia and infarction are key pathophysiological mechanisms of the lesions seen. Therapies that have been used include antiplatelets, anticoagulants, immunosuppressive agents, plasma exchange, and nicotine patches. Surgery is indicated in cases of intestinal perforation.