Leiomyomas are the most common benign tumors found in the esophagus. They are mostly solitary and multiple diffuse lesions are rare, occurring in only 2.4% of cases (1). We describe the case of a 13-year-old boy with a history of Alport syndrome who complained of progressive dysphagia and postprandial vomiting, and in whom diffuse leiomyomatosis of the esophagus was diagnosed. Chest PA showed mediastinal widening, and a barium study revealed diffuse esophageal wall thickening with dilatation, and obstruction at the level of the distal esophagus. Manometry showed increased pressure in the lower esophagus, and CT demonstrated diffuse thickening of the entire esophageal wall and an intraluminal mass in the distal esophagus. Follow-up CT three years later showed further esophageal wall thickening, as well as luminal narrowing. By means of distal esophagectomy, diffuse leiomyomatosis involving the entire esophageal wall and intraluminal mass was diagnosed.