Phaeochromocytomas are rare neuroendocrine tumours that produce symptoms through excess release of catecholamines. Treatment of choice is elective, complete surgical removal after pretreatment with alpha-adrenergic blocking drugs, to prevent dangerous haemodynamic fluctuations. In rare cases a 'catecholamine crisis' develops presenting with pulmonary oedema and circulatory shock. We report such a case of a patient with familial extra-adrenal phaeochromocytoma who successfully underwent emergency surgery. Pathophysiological mechanisms are discussed. Although pretreatment with alpha-adrenergic blocking drugs seems advisable in terms of morbidity and mortality, the concept is based on theory rather than clinical evidence. Surgical management of a catecholamine crisis is associated with high mortality rates. However, proof of better outcome by avoidance or discontinuation of emergency surgery is not available. Based on literature and on this case, we conclude that emergency surgery in phaeochromocytoma does not have to be structurally avoided and may be considered under life-threatening circumstances.