A 65-year-old man presenting with an esophageal lesion underwent esophagogastroduodenoscopy and computed tomography and an upper esophageal submucosal mass was found. Endoscopic ultrasound-guided fine-needle aspiration led to the diagnosis of mucosa-associated lymphoid tissue lymphoma of the esophagus. Positron emission tomography showed abnormal uptake in the internal iliac lymph nodes, and laparoscopic biopsy findings confirmed the diagnosis of esophageal cancer. The patient declined treatment of any kind and has been followed up for 39 months with no progression of the lymphoma.