Achalasia cardia is more common in adults between the ages of 30 and 60 years. It is relatively uncommon in children and very rare in infants. Only a few cases of infants with achalasia have been reported till now. It is a motility disorder of the esophagus due to a failure to relax the lower esophageal sphincter. The common clinical presentations in infants are regurgitation, vomiting, respiratory symptoms, and failure to thrive. This can be easily misdiagnosed as gastroesophageal reflux disease. Surgical management is the mainstay of treatment. Here, we present the case of a female newborn with symptoms suggestive of achalasia from day one of life and successfully treated with Heller’s esophagocardiomyotomy and Nissen’s fundoplication, following which the baby is asymptomatic and thriving well.