Biliary atresia is the most frequent cause of surgical jaundice in
the late neonatal age group. Congenital extrahepatic biliary atresia
and obstructive hydrocephalus is a rare association and not reported
earlier in literature. We report a 2-month-old baby who presented
with yellow discoloration of skin, high colored urine and clay colored
stools since 20 days of life. Head size was apparently looking
large with dilated veins over the scalp. The baby was diagnosed and
treated as extrahepatic biliary atresia that led to cholestatic jaundice
and cerebral aqueductal stenosis with hydrocephalus.