A rare case hyperlipoproteinemia in a 22-day-old infant who presented not only high blood levels of cholesterol and triglycerides but also an ocular manifestation described as lipemia retinalis. The fundus abnormality cleared as the lipid profile improved. Lipemia retinalis is an important and reliable parameter of high levels of chylomicrons and triglycerides and should be considered as a significant clue while diagnosing.