The incidence of colon ischemia has increased in recent years, and is associated with high morbidity and mortality. The typical presentations of colon ischemia include abdominal pain, bloody diarrhea, and in severe case, ileus, fever and peritonitis. Here, we document a rare case of colon ischemia presenting with subcutaneous and intramuscular emphysema of the thigh. A 76-year-old woman presented to the emergency department for left thigh pain for three days. Physical examination revealed tenderness without obvious crepitus, erythema or swelling over the left groin area and a soft abdomen without tenderness. Plain abdominal film showed abnormal gas formation at the left thigh and chest film demonstrated subphrenic free air. Abdominal computer tomography found sigmoid perforation causing left retroperitoneal abscess, and subcutaneous and intramuscular emphysema over the left pelvic and thigh region. During operation, irreversible ischemia from the terminal ileum through the cecum to the sigmoid colon with gangrene and retroperitoneal abscess were found. Total colectomy with end ileostomy and peritoneal toilet were performed. However, massive bloody ascites from abdominal drainage developed on the 13th day of admission. She later passed away due to hemorrhagic shock. In conclusion, emphysema of the thigh may rarely be caused by an intestinal lesion, such as colon ischemia. Clinicians should be alert of these unusual presentations to find the hidden underlying etiologies.