Cystic fibrosis (CF) is a hereditary disease of mucous and sweat glands, which affects the respiratory and gastrointestinal systems. Herein, we describe a 3-month-old girl with a history of recurrent episodes of urinary tract infections that required hospitalization. She was referred to our center at the age of three months, with massive gastroesophageal variceal bleeding. In physical examination, she had clubbing, hepatosplenomegaly, and mild ascites. Laboratory studies revealed high serum levels of liver enzymes and low level of Albumin. As of suspicious to CF, sweat tests were performed twice which confirmed the diagnosis of CF. Gastrointestinal bleeding due to gastroesophageal varices is a rare complication of CF, which could result as a consequence of hepatobiliary involvement of disease. Early diagnosis of CF could prevent severe complications and even death in this group of patients.