Cystic fibrosis is a common inherited fatal disease. As the life expectancy of affected individuals continues to increase with advances in disease management, this disease is no longer limited to the pediatric population. Currently, 40% of patients with cystic fibrosis are adults. In addition, patients may not present until adulthood and frequently have extrapulmonary symptoms. Abdominal manifestations are common and affect multiple organ systems. Hepatobiliary manifestations include fatty infiltration of the liver, gallbladder abnormalities, bile duct abnormalities, focal biliary fibrosis, and multinodular cirrhosis. Manifestations in the pancreas include acute pancreatitis, fatty replacement, calcifications, cysts, duct abnormalities, and carcinoma. Gastrointestinal manifestations include gastroesophageal reflux, peptic ulceration of the gastric and duodenal mucosa, distal intestinal obstruction syndrome, intussusception, appendicitis, fibrosing colonopathy, pneumatosis intestinalis, rectal mucosal prolapse, malignancies, and pseudomembranous colitis. Renal manifestations include nephrolithiasis, as well as secondary renal complications such as interstitial nephritis due to antibiotic therapy and amyloidosis. Awareness of these manifestations is important to successfully guide management of cystic fibrosis in adult patients.