The severe hypertriglyceridemia during pregnancy is a rare condition. It is a problem for diagnostic, prognostic and therapeutic. This dyslipidemia benefit from specific and effective treatments, but it is still poorly codified. Dietary is still the essential therapeutic, but fetal extraction should also be considered if the gestational age permits. Post-partum monitoring is required but etiologic thorough is not recommended if the triglycerides rate normalizes. The major complication of hypertriglyceridemia should be actively sought because of important maternal mortality rate.