Langerhans cell histiocytosis (LCH) refers a group of disorders of the reticuloendothelial system characterized by a proliferation of histiocytes, which includes eosinophilic granuloma, Lettere-Siwe diseases, and Hand-Schuler Christian disease. The clinical presentation of LCH depends on the site of involvement. It can range from multifocal lesions to a solitary lesion. Tissues characteristically involved in LCH are bone, skin, lung, liver, spleen, bone marrow, lymph nodes and the hypothalamic-pituitary region, although the involvement of other organs such as the bowel can occur. We experienced a case of external auditory canal stenosis in LCH of multiple organ involvement in a 28-year-old male, and report it with a review of the relevant literature.