Absent pulmonary valve syndrome is a rare congenital anomaly, usually seen in association with a ventricular septal defect. It has been reported to occur in 3–6% of cases of tetralogy of Fallot. Absence of the pulmonary valve results in a dilated main pulmonary artery, which can be seen as a cystic, pulsatile, paracardiac lesion on antenatal USG. Such a lesion, though rare, can easily be detected. We report a case of this rare anomaly which was present in association with a ventricular septal defect, tetralogy of Fallot, and dextrocardia. The case was detected at 26 weeks of gestation.
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