The frequency and severity of intestinal tuberculosis are decreased due to socioeconomic development, pasteurization of milk and more effective anti-tuberculous chemotherapy. But in recent year, HIV-infected patients are known to be at high risk of developing tuberculosis. The pathogenesis of intestinal tuberculosis is dependent on a immune state and nutritional status of the patient, the tuberculosis amount of sputum and tuberculosis toxicity. The diagnosis of intestinal tuberculosis is usually difficult because of non-specific clinical features and radiological signs. Chronic diarrhea in intestinal tuberculosis is able to produce a malnutrition, marasmus, kwashiorkor or the combined form. The treatment is anti-tuberculous chemotherapy for 12~24 months, but operation should be considered to intestinal perforation, obstruction, fistula formation and massive bleeding. The study about kwashiorkor in intestinal tuberculosis is rare in recent years, we should remind that tuberculosis is still prevalent disease in Korea. We report a ten year-old boy with ileocecal tuberculosis who presented with kwashiorkor, severe malnutrition with review of literature.