In this review, the function of the sphincter of Oddi (SO) is detailed in terms of normal motility, neural and hormonal control of SO function, coordination between gallbladder and SO motility, and correlation of motility of the SO and the duodenum. In addition, SO function tests, such as the morphine Prostigmin test (Nardi test), perfusion manometry, microtransducer manometry, and cholescintigraphy, are explained. Subsequently, the pathophysiology, diagnosis, and treatment of SO dysfunction, including SO stenosis and dyskinesia, are described and discussed in detail. SO manometry and endoscopic sphincterotomy are effective to treat SO dysfunction, but symptoms of the patient must be severe enough to justify these invasive procedures for diagnosis and treatment.