Acute pancreatitis due to hypertriglyceridaemia during pregnancy is a rare but severe clinical condition that may cause fatal results for both the mother and the foetus. Acute pancreatitis developed in a 37-year-old pregnant woman with familial hypertriglyceridaemia and diabetes mellitus in the 31st week of pregnancy. As intrauterine foetal death developed, the pregnancy of the patient was terminated. Additionally, insulin, octreotide and plasmapheresis with double membrane filtration were applied, and triglycerides rapidly decreased. After 24 hours, the level of triglycerides decreased from 9742 mg dL-1 to 432 mg dL-1. The patient was discharged from the intensive care unit at the end of 5 days and was discharged from the hospital after 32 days. The current article presents the successful treatment of severe hypertriglyceridaemia in a pregnant case