Chylomicronemia-induced pancreatitis is a potentially life-threatening condition that warrants emergency treatment. Most patients with this problem have an underlying genetic disorder of triglyceride metabolism which is superimposed on one or more forms of secondary hypertriglyceridemia, such as uncontrolled diabetes, alcohol intake, and excess dietary fat intake. Eruptive xanthomas, lipemia retinalis, and lipemic plasma are signs of chylomicronemia that may be identified by the clinician. Treatment of chylomicronemia-induced pancreatitis is focused on correction of the chylomicronemia as well as reversal of the factors that may be aggravating hypertriglyceridemia. Long-term therapy requires extensive lifestyle changes, often with the addition of triglyceride-lowering medications. A very low-fat diet, avoidance of alcohol, regular exercise, and sustainable weight loss are important components of the therapeutic intervention. Patients who are compliant with their treatment regimen can often avoid a recurrence of pancreatitis, but recurrent pancreatitis occurs in many patients.