A previously non-described cause of mitral regurgitation is presented. An asymptomatic 50-year old male who was casually diagnosed of mitral valve Barlow's disease underwent cardiac surgery due to severe mitral regurgitation. In the operating theatre, a longitudinal fissure of 1.5-2.0 cm length, along the posterior mitral leaflet, was found responsible for the insufficiency. This defect had features of a potential congenital origin and it was successfully repaired with direct suture. Whether it is an atypical mitral cleft, a variation of Barlow's morphology spectrum or a new congenital heart defect remains unclear.
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