Mitral valve prolapse (MVP) is still a clinical challenging problem. In this report, we review the main characteristics of this entity. Epidemiology of MVP, which relies on the diagnostic criteria adopted, and the incidence of complications, both arrhythmic and structural, are influenced by the characteristics of the population studied, which may lead to bias in data interpretation. Even the definition of MVP may differ according to the cardiologist's or cardiac surgeon's point of view. Usually, cardiologists define MVP as the protrusion of all or part of the mitral leaflets into the left atrium, independent of maintenance of coaptation. Therefore, using this definition, mitral regurgitation is considered as a complication rather than a diagnostic criterion. Arrhythmias, either supraventricular or ventricular, are other possible complications, mostly not life-threatening and associated with myxomatous degeneration of the valve. Diagnosis of MVP is based on echocardiography, which provides detailed anatomic and functional evaluation of the affected valve. Leaflet thickness and motion as well as presence and severity of mitral regurgitation can be assessed, with important diagnostic and prognostic implications. Echocardiographic evaluation of the mitral valve requires a systematic approach in order to define the leaflet/scallop involved and the mechanisms of mitral regurgitation. To this aim, three-dimensional reconstruction may add further insights into objective rendering of mitral valve pathology. Finally, surgical timing in mitral regurgitation due to MVP is an evolving issue and the likelihood of surgical repair is a crucial factor in the optimal timing of surgical intervention, especially in asymptomatic patients with severe mitral regurgitation.