Postural orthostatic tachycardia syndrome (POTS) has been recognized since at least 1940. A review of the literature identifies differences in the definition for this condition and wide variations in treatment and outcomes. This syndrome appears to describe a group of conditions with differing pathophysiology, which requires treatment tailored to the true underlying disorder. Patients need to be fully evaluated to guide treatment. Further research is required to effectively classify the range of underlying pathophysiology that can produce this syndrome and to guide optimal management.