Summary Acrodermatitis enteropathica is a rare disorder, caused by an inherited or acquired zinc deficiency. It is characterized by acral and periorificial dermatitis, alopecia, and diarrhea. We report the case of a 19 month old boy with typical lesions of the disease, which was confirmed by a serum zinc level of 13.3 μg/dl. The patient was given zinc sulfate at a dose of 1 mg a day and a zinc rich diet with which he improved significantly. A review of the literature is presented.