Familiar xanthomatosis hypercholesterolemia in homozygous or heterozygous (two or more defects in LDL receptors) forms are rare. The cholesterol levels are frequently above 700 mg/dL. The management of these patients includes odd procedures, such as ileal bypass to control of levels of cholesterol. We present a case of pregnancy in patient with familiar hypercholesterolemia (FH) and coronary and cerebral atherosclerosis who had undergone ileal bypass, five years prior. During pregnancy, there were no clinical signs or symptoms related to coronary or cerebral atherosclerosis and we did not observe obstetric complications. Nevertheless, the levels of cholesterol and triglyceride increased significantly to 1182 mg/dL and 807 mg/dL. Face the unknown prognosis of this clinical situation we decided to hospitalize the patient and to prescribe prolonged rest, dietary measures and specific therapy. This approach permitted her to reach the end of pregnancy without maternal and fetal complications. The patient was submitted to cesarean section by obstetric reasons. The newborn was healthy but his levels of cholesterol and triglycerides were respectively, 339 mg/dL and 301 mg/dL. The success of this case does not allow the change in the recommendation of avoiding pregnancy in patients with severe FH.