RESUMEN
INTRODUCTION: Histiocytosis X or Langerhans cell granuloma is a rare disease characterised by the proliferation of phagocytes which have normal cell markers. EXEGESIS: We report the case of a 44-year old woman with hypothalamo-hypophyseal Langerhans cell histiocytosis with genital and oral cutaneo-mucous involvements. After several non-active treatments, thalidomide began. Oral and genital lesions healed quickly. Recurrence of symptoms was observed six months after the treatment was stopped and a complete remission was obtained when thalidomide was reintroduced. The regression of hypothalam-hypophyseal granuloma infiltration was confirmed by MRI and the treatment was well tolerated by the patient. CONCLUSION: Cutaneo-mucous Langerhans cell disease as well as hypothalamo-hypophyseal histiocytosis may both benefit from a treatment with thalidomide.