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Achalasia cardia is defined as a neuromuscular disorder of the oesophagus with abnormal motility and failure of relaxation of the distal oesophagus. It is an uncommon but well-recognised entity in infants and children. However, achalasia in a preterm baby has not been previously described. We report the condition in a premature infant with unusual presentation, treated successfully with Heller's oesophagomyotomy and fundoplication.