We discuss an extremely unusual presentation of a 19-month-old child with cor triatriatum and an intact interatrial septum, who presented for the first time at the age of 16 months with wheezing and repeated lower respiratory tract infections. At surgery, a thick fibromuscular membrane with a 2-3 mm eccentrically placed orifice was identified, and following surgical resection of the membrane the child made an uneventful recovery. This case demonstrates the need for investigating children with "asthma" who do not respond to conventional medical management. A rare but potentially correctable underlying cause may be found.