Jaundice caused by cystic fibrosis is rare in the neonatal period. The reported child had jaundice, possibly due to cystic fibrosis, at 3½ weeks of age. This was followed by hepatomegaly, hyperbilirubinemia and abnormal levels of hepatic enzymes at 6 weeks of age. Complete resolution of clinical and laboratory evidence of liver disease occurred at 10 weeks of age, which suggests that temporary plugging may have caused the jaundice. Treatment with choleretics may be indicated.