The authors report the case of a 60 year-old woman patient with esophageal papillomatosis, revealed by slowly progressive dysphagia and digestive hemorrhage. Multiple warty tumors were found at endoscopy, starting at approximately 23 cm from the dental ridge, increasing in size into the lower esophagus where they were responsible for stenosis. Pathological examination demonstrated epithelial proliferation with lengthened papillae, hyperkeratosis, hyperacanthosis and severe dysplasia. No extra-esophageal papillomata were discovered. Subtotal esophagectomy was performed and pathological examination with immune markers suggested a human papilloma virus (HPV) infection. However, search for HPV DNA was negative. To our knowledge, this constitutes the fifth case reported in the literature. The principal problem posed by this rare disease is the possible association with and/or progression to carcinoma, the diagnosis of which may be difficult, and particularly, with verrucous carcinoma. With this diagnostic uncertainty in mind, the authors suggest total surgical removal of the esophagus in this situation.