Langerhans cell histiocytosis includes a broad spectrum of clinical diseases resulted from proliferation and tissue infiltration of differentiated langerhans cells. This term includes Letterer-siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma and congenital reticulohistiocytosis that are more common in children than adults. This entity has recently been classified into restricted and extensive forms according to the number of involved organs. We report here in a 41-year-old man who developed Langerhans histiocytosis and presented with seborrheic dermatitis-like skin signs and external ear polypoid lesion without other organ involvement.