Cricopharyngeal achalasia is an uncommon cause of dysphagia, especially in children. Congenital form is known in neonates and infants. In older children this disease has been reported in very rare cases and mostly in connection with neurological and muscular diseases. We present a case of a 12-year-old girl with a four-year history of dysphagia. Esophagogastroduodenoscopy, radiological contrast swallow study and esophageal manometric study confirmed the diagnosis of cricopharyngeal achalasia. The patient was successfully treated with dilatation of the upper esophageal sphincter. An initial attempt of dilatation appears to be a safe and effective option in the management of cricopharyngeal achalasia in children, and may prevent or at least postpone the need for myotomy. Following the diagnosis of cricopharyngeal achalasia, several autoimmune conditions were diagnosed. Our case report revealed that cricopharyngeal achalasia may occur in association with some other autoimmune conditions, and that autoimmunity may also play a role in cricopharyngeal achalasia itself. IzvlečekKrikofaringealna ahalazija je pri otrocih izjemno redek razlog za disfagijo. Poznana je kongenitalna oblika krikofaringealne ahalazije pri novorojenčkih in dojenčkih, pri starejših otrocih pa so to bolezen doslej opisali zelo redko in večinoma v povezavi z nevrološkimi in mišičnimi boleznimi. V prispevku prikazujemo primer 12-letne deklice s štiriletno anamnezo disfagije. Z ezofagogastroduodenoskopijo, radiološko preiskavo požiranja s kontrastom in ezofagealno manometrijo smo potrdili diagnozo krikofaringealna ahalazija. Bolnico smo uspešno zdravili z dilatacijo zgornjega ezofagealnega ustja. Začetni poizkus zdravljenja z dilatacijo se zdi varna in učinkovita metoda zdravljenja krikofaringelane ahalazije pri otrocih in lahko prepreči ali vsaj odloži potrebo po miotomiji. Po postavitvi diagnoze krikofaringealna ahalazija smo pri bolnici diagnosticirali več avtoimunskih motenj. Opisan primer prikazuje povezavo pojava krikofaringelane ahalazije z avtoimunskim dogajanjem in možnost, da bi lahko bil tudi avtoimunski proces eden od razlogov za pojav krikofaringealne ahalazije.