Syncope represents a frequent complaint in childhood and adolescence which is characterized by a spontaneous reversible loss of consciousness of short duration. The key point in the diagnostic evaluation is the differentiation between harmless neurally mediated syncope and syncope due to organic diseases. An exact medical history, physical examination and a resting echocardiograph frequently provide enough evidence to clarify this. Further diagnostic procedures are required whenever organic diseases are suspected (neurologic, cardiac, psychiatric, endocrinologic/metabolic syncope). When syncope due to organic diseases is suspected specific therapeutic strategies for treatment of the underlying disease are necessary. An excellent prognosis is reported in neurally mediated syncope, whereas among syncope due to organic diseases, cardiac syncope in particular shows a high mortality. The aim of this article is to focus on the evaluation of pediatric patients with syncope in terms of diagnostic procedures and treatment options.