Recurrent wheezing is a very common clinical ailment throughout infancy and childhood. The most common diagnosis in children with wheezing is asthma. However, some other causes should be considered in the differential diagnosis such as a congenital cardiac defect. We present a case of a four year old boy presenting with recurrent wheezing who was referred to our institution for cardiac evaluation. Severe mitral stenosis secondary to an anomalous mitral arcade was diagnosed by physical examination, chest X-ray, electrocardiogram and mainly transthoracic and transesophageal echocardiography. Anomalous mitral arcade is a rare congenital malformation of the mitral tensor apparatus which comprises the chordae tendineae and papillary muscles. This abnormal anatomy leads to increased filling pressure of the left ventricle, a retrograde post capillary pulmonary hypertension and interstitial aedema. The patient was referred to cardiac surgery and underwent a successful procedure with complete removal of the obstructive mitral lesion.