Multiple endocrine neoplasia, type II B (MEN II B) includes thyroid carcinoma, pheochromocytoma, ganglioneuromatosis and marfanoid habitus. Its short-term prognosis is determined by the tumour of the adrenal gland, whereas long-term prognosis is determined by the thyroid carcinoma. Often the well-defined but rare syndrome is diagnosed late. The MEN II B's therapy consists of thyroidectomy and adrenalectomy uni- or bilateral. The 5 year-rate of survival is 75%. Own observations are reported.