Intestinal fibrosis is a rare complication of chronic inflammation resulting from various etiologies, including surgery, abdominal radiation, and inflammatory bowel disease. Consequences of intestinal fibrosis include intestinal dysmotility, malabsorption, and obstruction. Patients with Lynch syndrome are predisposed to developing intestinal adenocarcinoma including in the small intestines which typically require intraabdominal procedures that expose them to fibrogenic triggers. Here, we present a rare case of duodenal fibrosis involving the sphincter of Oddi leading to malabsorption and gastrointestinal symptoms in a patient with Lynch syndrome requiring advanced endoscopy interventions.