Aim To study a family of hypertriglyceridemia with the metabolism of the lipids and apolipoproteins. Results The results showed that several mumbers of the family suffer from the hypertriglyceridemia. The serum, from the patients of the hypertriglyceridemia which was laid up icebox over night was turbid observed by naked eye. The level of the total cholesterol (TC) in the serum was normal and the values of triglyceride (TG) increased and the level of the high density lipoprotein cholesterol decreased. Although the concentrations of the apolipoprotien A_Ⅰ, AⅣ and B₁₀₀ had no significantly change, the concentration of the apolipoprotein E (apo E) had raised. The concentration of serum TG is positively correlated with that of serum apo E. Conclusions The family was appointed as a family of type Ⅳ hypertriglyceridemia. Experiments have shown that apo E metabolic disturbance may be one of factors that lead to hypertriglyceridemia. The reason why the family members of this familial hypertriglyceridemia case have high frequency to suffer liver cancer and appendicitis requires further research.