This review covers aspects of adrenal cortical tumours, phaeochromocytoma and extra-adrenal paragangliomas. Relevant clinical and epidemiological information is included. It is now known that about 30% of paragangliomas occur in a familial setting and these new aspects of the genetic background are presented. The main diagnostic problem in both groups of tumours is the recognition of malignant potential. The uses and limitations of multifactorial histological assessment in diagnosis and prognosis are discussed. Finally, data on the molecular changes associated with tumorigenesis and tumour progression are highlighted, and how this information may contribute in future to diagnosis and prognosis. © 2010 Blackwell Publishing Limited.