Langerhans' cell histiocytosis is characterized by abnormal accumulation or proliferation of cells, which is bearing a Langerhans' cell phenotype. It has been regerded as a malignant disease, but recent studies suggest that this disease has an immunological basis. The head and neck, especially temporal bone and ear, are frequent sites of the lesion. Recently, We experienced a case of Langergans' cell histiocytosis, manifestated with hearing loss in a 6-year old male patient. We report this with literature review.