Nonalcoholic fatty liver disease (NAFLD) has been reported in adults with hypothalamic or pituitary dysfunction, and some have progressed to end stage liver disease requiring transplantation. We report a teenager who initially presented with hypoxia due to intrapulmonary shunting, found to have NAFLD and cirrhosis associated with a hypothalamic tumor and panhypopituitarism. The NAFLD recurred very quickly after a successful liver transplant. Hepatopulmonary syndrome may be more common in cirrhosis associated with pituitary dysfunction than in other types of cirrhosis.