We report of a 29-year-old man presented with yellow-red, scaling papules on the skin of scalp, retro-auricular folds, inguinal, axillary, chest and abdomen. The lesions had a poikilodermatious appearance in some areas, such as trunk. The patient also had diabetes insipidus and skull osteolytic lesions. The diagnosis of langerhans cell histiocytosis (LCH) was suggested based on H and E staining of skin biopsy specimen. S-100 staining was positive in immunohistochemistry. Birbeck granules were seen in electron microscopic examination confirming the diagnosis of LCH. Considering the systemic involvement, he is a case of an extensive LCH in new classification and a case of Hand-Schuller-Christian in the old classification. The cutaneous lesions of the patients responded to oral thalidomide 200 mg daily after 2 months and did not recur after 6 months follow up.