Hypertrophic Pyloric Stenosis commonly presents with non-bilious progressive vomiting usually after a feed. The vomitingusually starts after third week with a range of symptoms onset from first week to fifth month of life. Less commonly itis associated with hyperbilirubinemia (unconjugated > conjugated) which subsides after surgical correction. We reporta case of Hypertrophic Pyloric Stenosis who presented early in fifth day of life with repeated vomiting after each feedand unconjugated hyperbilirubinemia. Ultrasonography revealed Hypertrophic Pyloric Stenosis without any evidence ofother cause of jaundice. Patient was treated conservatively, prepared for surgery, surgical correction done with Ramstedt’sPyloromyotomy following that vomiting and jaundice both subsided. Early presentation of HPS may suggest congenitaletiology and association of unconjugated hyperbilirubinemia should alert clinician for future Gilbert Syndrome
