Cystic fibrosis (CF) is a recessive autosomic disease with multiorgan, although predominantly pancreatic and pulmonary, involvement. Liver involvement is infrequent in children under the age of 5 years, but increases progressively with time. It is characterized by the development of focal biliary cirrhosis with eventual appearance of portal hypertension. During the last few years the more effective control of the pulmonary complications, which are the main cause of mortality, has led to an increase in the survival of these patients and thus the number of patients with CF and liver involvement is greater every day. In these cases, the prognosis is bad and most patients die in 4 to 5 years. Isolated liver transplantation is a recently proposed alternative for patients who have developed liver cirrhosis but who maintain acceptable pulmonary function. The case of a 14-years-old patient in whom liver transplantation was performed with good results after 8 months of follow-up is presented. Improvement in the nutritive state and pulmonary function was observed.