Overshadowed by Sino-pulmonary infections, Cystic Fibrosis (CF) commonly affects gastrointestinal organs because of secretory and motility dysfunction. Infrequently, these changes result in Distal Intestinal Obstruction Syndrome (DIOS), an increasingly diagnosed gastrointestinal entity in adult Cystic Fibrosis patients. We present a case 22-year-old male who presented to our hospital with right lower quadrant abdominal pain with suspicion of acute appendicitis and was subsequently diagnosed as DIOS. Our case highlights the importance of DIOS as one of the differential diagnosis of right lower quadrant abdominal pain in a patient with a CF, especially for physicians working at community hospitals which may not have a Cystic Fibrosis care program available.