Hypertrophic osteoarthropathy is an uncommon disease in the pediatric age group characterized by noninflammatory joint effusions, terminal digit clubbing, and radiographic evidence of periosteal new bone formation affecting the hands, feet, and distal limbs. The hepatopulmonary syndrome is also uncommon in childhood and presents as hepatic dysfunction, impaired arterial oxygenation, and intrapulmonary shunting. We report the case of a 17-year-old male with a history of liver transplantation at 4 months for biliary atresia who was initially diagnosed with juvenile rheumatoid arthritis but later developed features of classic hypertrophic osteoarthropathy. In addition, he was found to have the hepatopulmonary syndrome. It is important to consider hypertrophic osteoarthropathy as an imitator of juvenile rheumatoid arthritis, to recognize its known association with chronic liver disease, and to know that hepatopulmonary syndrome can occur in the setting of hypertrophic osteoarthropathy.