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Marfan syndrome (MFS) is a variable, autosomal dominant disorder of connective tissue whose cardinal features affect the cardiovascular system, eyes, and skeleton. The patient′s prognosis depends on the severity of cardiovascular complications and is mainly determined by progressive dilation of the aorta. If signs of MFS are recognized, it is important to refer to the correct health care professional for further testing to prevent associated complications. Hereby, we report a case of MFS who was unaware about the cardiac manifestations, thereby emphasizing the importance in identifying this potentially life-threatening condition in dental practice.