Lipemia retinalis is a rare ocular manifestation of certain types of hyperlipidemia. A case of a newborn with lipemia retinalis evaluated by laboratory and clinical findings is described. A creamy white appearance of all retinal blood vessels was demonstrated by color fundus photographs. The patient had elevated levels of triglycerides (29,000 mg/dL) and cholesterol (1,470 mg/dL). Lipid electrophoresis indicated hyperprebetalipoproteinemia. Type IV primary hyperlipoproteinemia was diagnosed.