Unilateral absence of pulmonary artery (UAPA) is a rare congenital anomaly that can present as an isolated lesion or may be associated with other Congenital heart diseases. It is usually detected in infancy or incidentally found on X ray chest in adulthood. The diagnosis of UAPA has rarely been made in a newborn. Only one case has been reported in newborn period from Belgium by F. Marchau who reported the rare occurrence of this anomaly in a 2-day old male baby. We report the rare occurrence of UAPA in a newborn female from India who presented with severe respiratory distress at birth with high cord blood TSH levels. Our case also reflects the importance of a high index of suspicion along with the value of X-ray chest and echocardiogram in the neonatal period in detection of this rare anomaly.