Primary crycopharyngeal achalasia consists of a failure of the crycopharyngeus muscle to relax at the appropriate time during the swallowing process in the abscence of other motor abnormalities. The principle treatment options for cricopharyngeal achalasia include either dilatation of the upper esophagus or surgical myotomy of the cricopharyngeus muscle. We report a primary cricopharyngeal achalasia case, who was diagnosed early in 1st week of life and treated successfully by balloon dilatation, and discuss the treatment options for this very rare disease. The presented case is the first successfully treated newborn case during the neonatal period in the literature with respect to balloon dilatation.