Although severe hypertriglyceridemia is rarely seen in pregnancy. it may result in acute pancreatitis leading to increased morbidity in both nother and fetus. Restriction of dietary lipid and lipid-lowering medications are the cornerstones of the treatment. Experiences with plasmapheresis are limited. Herein, a pregnant woman with hypertriglyceridemia who was given preconceptional counseling and medical therapy and kept under close observation since the early weeks of gestation, succesfully managed with dietary lipid restriction and especially with serial plasmapheresis is discussed. Her previous pregnancy was remarkable with fetal demise due to acute pancreatitis at 24th weeks of gestation. It is important that the clinicians have a clear understanding of the normal lipid profile during pregnancy, the clinical picture, the potential complications, available treatment options of hypertriglyceridemia particularly during pregnancy.