It is uncommon for a patient to have 2 different diagnoses contributing to neonatal cholestasis and poor growth. We present a 2-month-old female with extrahepatic biliary atresia status after Kasai procedure at 4 weeks old presenting with persistent neonatal cholestasis. The patient was admitted for intolerance of oral feeds, concern for cholangitis and Kasai failure, and nutritional optimization. She was found to have genetic testing positive for 2 rare cystic fibrosis transmembrane conductance regulator mutations and pancreatic insufficiency consistent with a possible diagnosis of cystic fibrosis–related disease. We discuss the implications and management considerations in a patient with both biliary atresia and cystic fibrosis.