Langerhans cell histiocytosis (LCH) is a rare systemic disease caused by proliferation of mature histiocytes; its association to histiocyto fibroma is rarely reported. It rarely affects adults. We report a case of systemic LCH, in an adult patient with osteolytic lesion causing a fistula between the left nasal cavity and hard palate, involving the bone, lung, lymph node and associated to multiple histiocyto fibroma. The patient was operating for a fistula, and he was treated by chemotherapy and corticosteroids. Langerhans´ cell histiocytosis is a rare case, especially in adult patient. The diagnosis was based on histological and immunohistochemical analyses. This patient was treated by steroids and chemotherapy.