The internal anal sphincter, the smooth muscle component of the anal sphincter complex, has an ambiguous role in maintaining anal continence. Despite its significant contribution to resting anal canal pressures, even total division of the internal anal sphincter in surgery for anal fistulas may fail to compromise continence in otherwise healthy subjects. However, recently reported abnormalities of the innervation and reflex response of the internal anal sphincter in patients with fecal incontinence indicate its significance in maintaining continence. The advent of sphincter-saving surgery and restorative proctocolectomy has re-emphasized the major contribution of the internal anal sphincter to resting pressure and its significance in preventing fecal leakage. The variable effect of rectal excision on rectoanal inhibitory reflex has led to a reappraisal of the significance of this reflex in discrimination of rectal contents and its impact on anal continence. Electromyographic, manometric, and ultrasonographic evaluation of the internal anal sphincter has provided new insights into its pathophysiology. This article reviews advances in our understanding of internal anal sphincter physiology in health and disease.