RESUMO
A 8-year-old schoolgirl from West Africa with no previous pathological history was admitted to the haematology department of the Brazzaville University Hospital for the management of cervical adenopathy. The diagnosis of sinus histiocytosis or Destombes-Rosaï-Dorfman disease was retained and the patient was treated with PO corticosteroids (methylprednisolone 32 mg/d then 16 mg/d). Given the rarity and uncertain aetiopathogeny of this syndrome, treatment is poorly codified. It includes corticosteroid therapy, immunomodulators and sometimes chemotherapy, radiotherapy or surgery, indicated in case of clinical manifestations of local organ compression. The disease may regress spontaneously. Its benignity does not justify systematic treatment in the absence of complications.