Pemphigus vulgaris (PV) is an autoimmune blistering disease associated with symptomatic involvement of the skin and mucous membranes. Most patients present with fragile, bullous oral lesions. Though the esophagus is lined with stratified epithelium, PV rarely involves the entire esophagus1. Prior study has shown that patients with esophageal involvement manifest as dysphagia (57%) and odynophagia (21%), whereas hematemesis is seen in only 3.5% cases2. Here we describe an atypical case of PV isolated to the esophagus wherein endoscopy was critical in diagnosis. A 48 year-old male with no significant medical problems presented with a 2-month history of dysphonia and pharyngeal discomfort with associated 20-pound weight loss. He described severe, sharp pain localized to the posterior tongue and pharynx, worse with swallowing. He had tried Azithromycin without improvement in symptoms causing him to seek further care. His exam showed injected conjunctiva, palatal and pharyngeal erythema with white exudates on the tongue. He was started on Nystatin for presumed fungal esophagitis, laryngoscopy showed oropharyngeal white patches, but with negative cultures. Initial EGD showed friable, erythematous mucosa with severe esophagitis; biopsies were consistent with Herpes Simplex, thus the patient was started on Acyclovir. He had persistent symptoms on week 2 of Acyclovir treatment. This prompted a repeat EGD which revealed circumferential erythema, mucosal sloughing involving almost the entire esophagus to the gastroesophageal junction (Figure 1). The biopsies showed suprabasilar acantholysis with papillomatous features and scattered dyskeratotic cells most consistent with PV (Figure 2). Subsequently he was seen by dermatology, who initiated steroid therapy resulting in a dramatic response.Figure 1Figure 2This case highlights the importance of endoscopy in the diagnosis of primary esophageal PV, especially in the absence of skin lesions. Dysphagia and odynophagia are common presenting symptoms in esophageal PV, similar to infectious esophagitis. Physical exam is often nonspecific resulting in an incorrect diagnosis and improper treatment. Evaluation with upper endoscopy is prudent in patients with unclear diagnosis in order to properly guide management.