Phaeochromocytomas are rare in childhood. Only 5 to 10% of these neoplasms become manifest during the first two decades of life. Before puberty the incidence of phaeochromocytoma in boys is twice than in girls. Phaeochromocytoma is successfully treated only by surgery. A standardised concept of therapy is important; it should comprise the following points: 1. Diagnosis as early as ever possible. 2. Improved diagnosis of localisation via ultrasound, scintigraphy and computed tomography. 3. Standardised perioperative drug therapy in cooperation by the paediatric surgeon with the anaesthesiologist and paediatrician. If these guidelines are observed, the prospects of cure are 95-100% in case of chromaffin tumours.