Menetrier's disease (MenD) is a rare, acquired disorder associated with giant gastric folds along with protein-losing enteropathy, hypo- or achlorhydria, and histologic features of massive foveolar hyperplasia. Little is known about the etiology, clinical features, or epidemiology of this disorder, including risk of gastric cancer. We investigated outcomes and characteristics of patients with MenD, including development of gastric cancer and survival times.We performed a case-control study of all MenD cases (n=76; mean age 56±45 years; 59% male; mean body mass index, 24) diagnosed at Mayo Clinic, Rochester, Minnesota, from January 1975 through 2005. Diagnosis of MenD was based on combination of clinical, endoscopic, radiologic and histologic features. Patients with dyspepsia who underwent gastric biopsy analysis were included as controls. We obtained demographic, clinical history, laboratory, imaging, histopathology, and follow-up data from medical records. Clinical characteristics of MenD analyzed using descriptive statistics. Kaplan-Meier method used to estimate overall survival in cases.Clinical features found in a significantly higher proportion of patients with MenD than controls included vomiting, abdominal pain, postprandial fullness, and weight loss of 10 lbs or more. Smoking was associated with MenD (P=.002 vs controls), but not alcohol use. Infection with Helicobacter pylori was not associated with MenD (2.6% of patients vs 4.0% of controls; P=1.00). There was no significant difference between patients with MenD vs controls in proportions with inflammatory bowel diseases. Gastric cancer developed in 8.9% of patients with MenD by 10 years after MenD diagnosis vs 3.7% of controls over the same time period (P=.09). Of patients with MenD, 72.7% and 65.0% survived for 5 and 10 years, respectively, compared with 100% of controls.