Postural tachycardia syndrome (POTS) is a chronic condition with frequent symptoms of orthostatic intolerance or with sympathetic activation and excessive tachycardia while standing, without significant hypotension. The aim of this review was to discuss the pathogenesis and to outline the diagnosis and treatment guidelines. We conducted a literature review of articles published up to 2017, in following databases; PubMed, and Embase investigating postural tachycardia syndrome in children. We restricted our search to only English published articles with human subjects concerning children under 16 years. POTS is a disorder of the autonomic nervous system that could create considerable disability amongst previously healthy people. Patients with POTS show a HR rise of ≥ 30 bpm within 10 min of standing (or greater in kids), are typically hyperadrenergic, and tend to have a reduced blood volume. The pathophysiology of POTS is complex and the result of a variety of separate systems producing a common pattern of signs. The specific pathogenesis of POTS has yet not been completely clear. A variety of uncommon factors might be involved in the pathogenesis. Selecting the correct therapy according to the detailed pathogenesis could absolutely enhance the efficiency of medicine. Treatments targeting the hypovolemia and the excess sympathetic nervous system activation may assist eliminate symptoms.