This is a case report of a 35-year-old secundigravida woman with recurrent episodes of hypertriglyceridaemia-induced acute pancreatitis during pregnancy, which is a rare but serious complication in pregnancy. The woman was normal weight, and she was not known with any metabolic disorders, but she had idiopathic splenomegaly, which might be explained by the high triglyceride levels. Later genetic analysis revealed a lipoprotein lipase gene mutation known to cause hypertriglyceridaemia. This shows, that it is important to consider genetic aetiology in normal weight pregnant women with hypertriglyceridaemia and pancreatitis.