Mitral arcade (MA) is a rare congenital anomaly of the mitral valve and its tensor apparatus. This condition has been reported primarily in children younger than 3 years of age. MA is characterized by elongated papillary muscles connected to each other and to the tip of anterior mitral leaflet by a bridge of fibrous tissue. Because of progressive mitral regurgitation and/or mitral stenosis, survival into adulthood is rare. Not much is known about MA in the adult population. In this review we address the demographic features, clinical presentation, diagnosis, treatment, and outcome of this congenital anomaly in adult patients.