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Hypohidrotic/anhidrotic ectodermal dysplasia (H/AED) is a rare congenital disorder that involves the epidermis and one or more of its appendages. Atopic dermatitis (AD)-like eczema in H/AED has been reported by several authors. In this study, a 13-year-old Korean boy presented to the Department of Dermatology of Korean Medicine with persistent, pruritic, AD-like eczema throughout the body. The eczema had persisted from early childhood (1 year-of-age), and the boy had occasionally been prescribed topical steroids and anti-pruritic agents. Before his visit, the patient's skin lesions had deteriorated and he had considered using alternative medicine. The patient was hospitalized for 5 days; during which time he was treated using twice-a-day acupuncture Moreover, we prescribed the patient modified Danguieumja on the basis of his blood-heat and wind-dryness patterns. To assess the efficacy of the treatments, the severity of symptom was evaluated using the Scoring of Atopic Dermatitis (SCORAD) index. Treatment safety was also assessed by laboratory tests on the admission and discharge days. The results showed that the patient' SCORAD scores decreased continuously. The total SCORAD scores were 68.5, 38.2, and 38.1 on admission, discharge, and follow up visit day respectively. Subjective symptoms and pruritus had also subsided. There were no reported adverse events during hospitalization, or abnormalities observed on AST, ALT, BUN and creatinine testing. It seems acupuncture and herbs may serve as an alternative for H/AED patients, however, we must concede that no direct evidence is available at present to explain the efficacy of the acupuncture and herbs. [ABSTRACT FROM AUTHOR]