In a patient with progressive dysphagia, postprandial vomiting, and a history of Alport syndrome, barium and manometric studies had been interpreted as consistent with achalasia, but a subsequent computed tomographic (CT) scan of the thorax was suggestive of a lower esophageal intramural mass. Multiple leiomyomas of the esophagus were later proved at thoracotomy. Differences between adult and pediatric leiomyomas and the association of leiomyomas with Alport syndrome are discussed.