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We report a 2-month-old male baby who presented with yellow discoloration of skin, high-colored urine, and intermittent clay-colored stools since 15 days of life. Head size was apparently looking large with dilated veins over the scalp. The baby was diagnosed and treated as neonatal herpes simplex virus hepatitis that led to cholestatic jaundice and cerebral aqueductal stenosis with hydrocephalus. It is a very rare association and less reported earlier in the literature.