We describe the case of a 72-year-old male patient who was suffering from Pagetoid spread to the esophageal mucosa from adenocarcinoma located in the distal esophagus and proximal stomach. Esophageal Paget's disease is rare. The histogenesis of extramammary Paget's disease has remained controversial. In the majority of cases, it originates from a primary esophageal tumor. In this report, we describe a patient who was suffering from esophageal adenocarcinoma and discuss the origin of extramammary Paget's disease in the light of the literature.