The patient was a 22-year-old woman who had presented in early childhood wi
th gastroesophageal reflux and who subsequently underwent surgery. It was c
ommented upon by the surgeons at that time that the esophagus was abnormall
y thickened. The patient subsequently presented during her first pregnancy
with a vulval tumor, which proved histologically to be a leiomyoma. She was
also found to have a grossly dilated esophagus and was thought to have ach
alasia. However, endoscopic ultrasound imaging showed gross hypertrophy of
the mid- and distal esophageal wall, with only mild symptoms of dysphagia,
which had been long-standing. The appearance of the esophagus was consisten
t with diffuse esophageal leiomyomatosis. In view of the associated vulval
leiomyoma, the patient demonstrated esophagovulvar syndrome.