Congenital vallecular cyst is an uncommon, benign but potentially dangerous condition causing respiratory distress and stridor. It is associated with sudden upper airway obstruction resulting in death due to its anatomical location in neonates and infants. We reported a rare case of 2 months old male infant presented with respiratory obstruction with failure to thrive with polydactyly (rarest finding) and managed timely with appropriate surgical marsupialization.