Postural tachycardia syndrome (PoTS) is a frequent polymorphic clinical syndrome, poorly known and, as a result, underdiagnosed especially in adolescents. It is a form of dysautonomia, but its exact physiopathology remains elusive. It is characterized by heterogeneous symptoms that accompany a disproportionate tachycardia upon the upright position. It can significantly impact adolescents' quality of life. Only a Schellong test is useful to make the diagnosis; however additional testing is frequently performed in order to exclude conditions that may mimic, exacerbate or impact management. Treatment in PoTS is primarily symptomatic. The main goal is to restore the patient's condition as quickly as possible. Its management is multidisciplinary and must involve the parents. The course of the syndrome is sometimes marked by relapses, but the prognosis is mostly favorable.