Congenital cricopharyngeal achalasia (CCA) is a transient disorder of the swallowing mechanism. Although the clinical picture of this rare entity has been clearly described, the treatment remains controversial. Four cases of CCA are presented. Gastrostomy was performed in two patients initially or following short-term conservative treatment including gavage feeding and continuous semiupright positioning, because of distressing respiratory problems. Two other patients were initially treated conservatively for 4 weeks and 6 months, respectively; the latter had undergone cricopharyngeal myotomy elsewhere. Gastrostomy was also eventually performed in these patients because they failed to thrive and respiratory symptoms and feeding difficulties persisted despite conservative treatment. All of our patients gained weight satisfactorily and respiratory symptoms subsided following gastrostomy. One patient died 4 months after the gastrostomy due to neurological problems. We recommend performing gastrostomy initially after the diagnosis of CCA is established in order to ensure satisfactory feeding during the spontaneous recovery period of this disorder, which can be unexpectedly prolonged.