Four cases of eosinophilic granuloma involving the female genital tract are reviewed. The disease may remain localized, but more often involves other organs. Of particular interest was the association of the disease with diabetes insipidus in three patients. One of the patients with diabetes insipidus was successfully treated with radiotherapy to the hypothalamic area as soon as the symptoms of the disease appeared. Genital lesions may respond to radiotherapy. Chemotherapy is to be used for disseminated disease. Treatment of this condition requires studying the extent of the disease and establishing early treatment, as with any malignant lesion.