The safe anaesthetic management of a child with a phaeochromocytoma requires an understanding of the pathophysiology of the disease, together with a thorough knowledge of its pharmacology, in order to avoid or minimize the potentially harmful cardiovascular changes that may occur during anaesthesia. Although there is a considerable amount of information on the management of the adult with phaeochromocytoma, much less has been written concerning children with the disease. Children differ significantly from adults in the incidence, location, presentation and management of this condition and these differences are discussed here together with some of the more controversial issues of management.