BACKGROUND AND METHOD: The paper reviews inheritable endocrine tumour syndromes based on recent literature and the experience of the authors. RESULTS AND CONCLUSIONS: A number of inheritable endocrine tumour syndromes are caused by mutations in proto-oncogenes or tumour suppressor genes. These include multiple endocrine neoplasia (MEN), familial non-MEN neoplasia, Von Hippel-Lindau's disease and familial medullary thyroid carcinoma, pheochromocytoma and paraganglioma. These conditions are rare, but since inheritance is dominant with a near-complete penetrance, a number of carriers with a high risk of developing tumours are regularly found in the families of affected individuals. Identification of carriers with a high risk of developing tumours gives the opportunity to cure disease manifestations at an early stage and to avoid life-threatening complications. Family members who are not carriers can be excluded from follow up. DNA analyses of the most common disease genes are now performed in genetic laboratories in Norway.