Hereditary thyroid carcinomas are present in about 5% of differentiated (DTC) and 25% of medullary thyroid carcinomas (MTC). They are part of a multiorgan tumour syndrome (e. g. FAP Gardner's syndrome with DTC and MEN 2 syndrome with MTC) or confined to the thyroid gland. Hereditary thyroid carcinomas typically show multifocal growth and occur in young patients. Due to germ cell mutations as the underlying cause of disease, partial thyroidectomies that may be justified in early sporadic carcinomas are not indicated in this type of tumours. In the case of hereditary DTC, the genetic basis of the disease has been demonstrated only in syndromatic tumour variants. In most nonsyndromatic cases, specific genetic alterations have not yet been identified. In both types of hereditary DTC, prophylactic thyroidectomy is not warranted due to the favourable prognosis of tumours that do not differ from sporadic ones. Point mutations of the RET proto-oncogene have been known for 15 years to be the genetic basis of hereditary MTC. Recently several new mutations were discovered; however, final conclusions regarding their clinical significance are not possible at present. Basically it has been shown that the clinical aggressivity of tumour development follows a genotype-phenotype correlation (risk groups 1-3). However, in mutations of all risk classes there exists a wide spectrum of different stages of hereditary C-cell disease in individual risk groups. Regarding time and extent of prophylactic thyroidectomy (without or with lymph node dissection) a combined molecular-biochemical concept including the use of pentagastrin-stimulated calcitonin values is therefore recommended.