Acrodermatitis enteropathica, an autosomal recessive disease, usually presents with severe acral and circumorificial dermatitis, diarrhea, alopecia, intercurrent bacterial infection during early infancy, and is eventually fatal if left untreated. We report a case of acrodermatitis enteropathica in a 2-month-old male infant who presented with chronic diarrhea not responsive to conventional therapy and developed disseminated intravascular coagulation (DIC). He showed the characteristic eczematoid skin lesions, chronic diarrhea, failure to thrive, and low serum zinc concenturation. Zn2+ was administered with dramatic improvement of skin lesions, DIC and diarrhea. He rapidly catched up normal growth and development on continuing zinc supplementation.