Eosinophilic esophagitis is a chronic immune-mediated disease characterized by infiltration of the esophageal mucosa with eosinophils and concomitant esophageal dysfunction. Though there are well-described associations between certain chronic inflammatory conditions and venous thromboembolism, there have been no reports of venous thromboembolism occurring in eosinophilic esophagitis. We report the case of a 33-year-old man with severe eosinophilic esophagitis resulting in recurrent esophageal strictures who was unresponsive to oral viscous budesonide therapy, and who developed an isolated pulmonary embolism in the absence of risk factors for venous thromboembolism. We then discuss potential mechanisms for venous thromboembolism in eosinophilic esophagitis, such as inflammation-mediated hypercoagulability, hypereosinophilia, and immunoglobulin E-mediated platelet activation.