Ménétrier disease (MD) is characterized by enlarged gastric folds with associated protein losing gastropathy. In children it is a rare and self-limited cause of protein losing gastropathy. We report a case of a 2-year-old male who presented with prolonged, refractory emesis and peripheral edema. Workup revealed severe hypoalbuminemia, hypoproteinemia, iron deficiency anemia, and high stool alpha-1 antitrypsin. Hepatic protein synthesis was normal with no urinary protein loss. Endoscopy showed antrum sparing, severe erosive gastritis in body and fundus, characteristic of MD. Histologic examination displayed inflammation with eosinophilia, foveolar hyperplasia, atrophic oxyntic epithelium, and rare CMV inclusions. Patient received antiviral therapy, intravenous albumin, diuretic and was discharged on high protein diet. Follow-up revealed clinical recovery, with endoscopy and histology showing normal gastric mucosa throughout the stomach. It is important to remain vigilant of this condition in pediatric population and to include it in the differential diagnosis in cases of protein losing gastroenteropathy.