Intraabdominal fibromatosis is an uncommon neoplasm. The aggressive nature of these tumors and the potential for major morbidity secondary to resection can present a difficult surgical dilemma. A 9-year-old boy presented with anemia and vomiting. He had esophagogastric fibromatosis diagnosed and underwent distal esophagectomy, total gastrectomy, Hunt-Lawrence jejunal pouch, and Roux-en-Y esophagojejunostomy. This report describes the successful surgical management of an esophagogastric fibromatosis for the first time in the English-language literature. The management of this tumor is discussed with particular regard to the very unusual clinical presentation.