VACTERL association is sporadic and nonrandom, with co‑occurrence of various congenital malformations in a single patient due to defect in structures derived from embryonic mesoderm. The VACTERL association with pulmonary agenesis is very rare; few cases have been reported only in neonates till now in the past, as most babies could not survive longer. However, we present a rare case of VACTERL association with left pulmonary agenesis in a 9‑month‑old infant.