Regarding the patient's care, a multidisciplinary consultation involving gastroenterologists and gastrointestinal surgeons was carried out. The patient is scheduled for esophagectomy.

Esophageal leiomyomatosis is a rare hamartomatous disorder. It predominantly affects children and young adults with female predilection. It may occur in isolation or in association with Alport's syndrome and visceral leiomyomatosis. This patient didn't have any other complications of the ocular, hearing or renal systems excluding Alport syndrome. However, esophageal-vulvar syndrome is a possibility, which is characterized by leiomyomata of both the esophagus and female genital system, and often occurs in young adult females.

Esophageal and anorectal leiomyomatosis association is exceedingly rare. It's characterized by benign smooth muscle cell proliferation in the esophageal and anorectal walls, respectively.

Leiomyomatosis mainly affects the middle and distal one-third of the esophagus. It may extend into the cardia and fundus of the stomach in approximately 80% of cases. The entire esophagus is involved in only 35% of cases ¹.

Management depends on clinical symptoms:

• asymptomatic patients: clinical and endoscopic follow-up is satisfactory

• patients with severe and progressive dysphagia should undergo total esophagectomy, with the use of gastric or colonic conduit ¹