Verruciform xanthoma is a distinctive lesion of oral mucosa and genital skin. It can be solitary or multifocal, as well as sporadic or associated with inflammatory, autoimmune, immunodeficient, metabolic, neoplastic, or congenital diseases. To our knowledge, it has not yet been described in the esophagus. The case of a 61-year-old man suffering from primary non-Hodgkin lymphoma of the testis is presented. Two years after initial diagnosis, mediastinal adenopathies were disclosed. Fractioned radiotherapy was administered; 3 years later, verruciform xanthoma of middle third of the esophagus was endoscopically resected. Histologically, the lesion showed acanthotic squamous mucosa infiltrated by neutrophils. Papillae were packed with foam cells that were positive for CD68 and vimentin antibodies. Verruciform xanthoma is a condition observed exclusively in squamous epithelia. From our viewpoint, physical agents play a preponderant role in the etiology, although viral agents may occasionally be involved in the development of this enigmatic lesion.