Hypovolemic and hemodynamic disorders，autonomic nervous dysfunction and hyperadrenergic state are recognized pathogenesis of postural tachycardia syndrome（POTS）. According to this，POTS can be divided into three types：hypovolemic POTS，neurogenic POTS and hyperadrenergic POTS. Autoimmune abnormalities，changes in hormone levels，and gene mutations may also cause POTS. In addition，some patients have joint overactivity syndrome，functional gastrointestinal disorders，Ehlers Danlos syndrome，mast cell activation disorders and other diseases.Therefore，POTS is a complex clinical syndrome that may involve multiple mechanisms.This article aims to offer clinicians an improved understanding of the pathogenesis of pediatric POTS through a comprehensive review of this disease.