ＬＰＬ绝对缺陷造成典型的家族性高乳糜微粒血症 ,罹患此症妇女罕见妊娠。本文报告 1例 18岁初产妇为与生俱来的ＬＰＬ基因缺陷 ,高乳糜微粒血症 ,其甘油三酯 (ＴＧ)为 12 9ｍｍｏｌ/Ｌ ,其中约 90 %为ＣＭ。对她治疗措施为低脂饮食。脂肪供 13 3%热卡 ,且含中链脂酸 (ＭＣＴ) LPL absolute defects caused by the typical familial hyperchylomicronemia, women suffering from this rare pregnancy. This paper reports a case of 18-year-old primipara was born with LPL gene defects, high chylomicronemia, triglyceride (TG) of 12 9mmol / L, of which about 90% of CM. Her treatment for low-fat diet. Fat for 13 3% heat card, and contains medium-chain fatty acid (MCT)