Amyloidosis is a disorder characterized by deposition of abnormally folded proteins in the extracellular space of various tissues and organs, possibly leading to their dysfunction. In the majority of cases, amyloidosis presents with systemic involvement including the gastrointestinal tract; however, localized gastroduodenal amyloidosis is rare. We report a case of gastroduodenal amyloidosis in a 36-year-old male with multiple comorbidities who presented with right upper quadrant abdominal pain. Reports of gastroduodenal amyloidosis and other relevant literature were also reviewed and discussed alongside this case.