Histiocytosis X rarely involves the thyroid gland and occurs as one of
the unusual manifestations of systemic diseases, Diagnosis is based o
n the identification of the Langerhans' cells in an appropriate backgr
ound, We report a case of histiocytosis X confined to the thyroid glan
d that represents the primary manifestation of the disease, A careful
histological, immunohistochemical and ultrastructural study has been p
erformed and problems of differential diagnosis are discussed.