BACKGROUND: Benign tumors of the esophagus are uncommon (less than 0.5% of all tumors) and fibrovascular polyps (FVP) comprise a small subset of this category. Establishing the diagnosis of FVP can pose a clinical challenge. METHODS: We present an atypical case of this unusual condition with endoscopic mucosal resection (EMR) as a diagnostic and therapeutic approach. We reviewed the presentation and management of our index case, then examined the Mayo Clinic electronic clinical database from 1994-2003, using the key words “fibrovascular”, “polyp”(benign/malignant), “lipoma”, “fibroma”, “hamartoma”, and “esophagus.” This retrospective search revealed 4 other FVP cases. RESULTS: Our index case was a 54 year old man who presented with a 3 month history of dysphagia and weight loss. Esophagogastroduodenoscopy (EGD) revealed an exophytic esophageal mass lesion in the distal esophagus measuring 9 cm in length. At endoscopic ultrasound (EUS), the lesion appeared to be confined to the esophageal mucosa. In addition, numerous malignant-appearing perigastric lymph nodes were detected. EUS directed biopsies were non-diagnostic. The degree of dysphagia and the lymphadenopathy (LAD) prompted us to proceed with EMR for both diagnostic and therapeutic purposes. Two large pieces of the mass lesion were removed with histologic features of a “giant FVP,” and his symptoms of dysphagia were transiently relieved for 6 months. In the other 4 cases (2 male, 2 female), FVP had the following characteristics on EUS: sizes ranged from 5.3 to 10 cm; 4/4 were located in the proximal esophagus; 2/3 had blood vessels visualized, one within the polyp; and 0/3 had evidence of LAD. The 4th EUS was terminated early due to airway obstruction by the FVP. All initial biopsy results (4/4) described nonspecific inflammation or esophagitis, consistent with reported routine biopsies of FVP showing a combination of normal epithelium, benign fibrous or fatty tissue, and nonspecific inflammation or esophagitis. Of these 4 cases, one was removed endoscopically with a needle knife device, the other 3 surgically excised with one recurrence. CONCLUSIONS: EUS can provide useful information regarding tumor size, density, vascularity, and stalk location but may not provide sufficient tissue for a diagnosis of a FVP. Malignant appearing LAD may be part of the spectrum of EUS findings, a feature not previously noted in the literature. EMR may help facilitate the decision-making process and provide therapeutic benefit.