We report about a boy with Barth-syndrome (X-chromosomal cardioskeletal myopathy, neutropenia and abnormal mitochondria) and postinflammatorial pharyngeal stenosis. After a pseudomembranous pharyngitis he developed a dyspnea and an inability to swallow with a resultant aspiration pneumonia. After endoscopic diagnosis of scarred pharyngeal stenoses the child required a tracheostomy and a percutaneous endoscopic gastrostomy tube. Then the boy underwent transoral pharyngoplastic surgery. Thirty five months after pharyngoplasty transoral nutrition was feasible. The tracheostomy was closed and the gastrostomy tube was removed. Throughout a 40 month period of observation the child had no more complaints relating to the minor residual pharyngeal stenoses.