We describe a case of submitral aneurysm in a 40-year-old man, which was distinctive in its anatomy and pathophysiology. The patient had a congenital submitral aneurysm located in the anterolateral part of the mitral annulus, complicated by dissection of the left atrial wall, severe mitral regurgitation, and compression of the left circumflex artery. He was managed successfully with aid of a comprehensive preoperative evaluation with all available imaging guidance and methodical surgical techniques, resulting in exclusion of the aneurysm, mitral valve replacement, and a graft to the left circumflex artery.
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