Pregnancy in HoFH females is associated with further elevation of already markedly elevated low density lipoprotein cholesterol (LDL-C) levels, particularly if lipid-lowering therapy is discontinued, placing the mother and fetus at increased cardiovascular risk. Lipoprotein apheresis is the current recommended treatment for pregnant HoFH patients. However, this is costly, time consuming, and is not available in many countries. Alternative treatment strategies to control hypercholesterolaemia during pregnancy in HoFH patients are necessary. This study was a retrospective review of 39 pregnancies from a cohort of 20 genotypically confirmed female HoFH patients. No maternal cardiac complications or deaths occurred during the pregnancies or during the first year postpartum. Twenty five pregnancies were exposed to lipid-lowering therapy, of which 18 were exposed to statin therapy, just prior to or during the pregnancy. Thirty three (84%) pregnancies carried to term, 3 (8%) premature deliveries and 3 (8%) miscarriages were observed. Complications associated with pregnancy in these HoFH patients, did not differ from those reported during pregnancies of otherwise healthy woman. HoFH is a severe disease impacting significantly on life expectancy. However, for many females with HoFH, despite the high cardiovascular risk, pregnancy is not uncommon. In resource poor settings and when LA is not available, lipid lowering therapy, particularly statin therapy during pregnancy, appears to be safe for both mother and fetus and is an acceptable alternative for LDL-C reduction in these high risk patients. •Statin exposure during pregnancy might not be as detrimental to the fetus as previously thought.•Pregnancy complications in homozygous familial hypercholesterolaemia patients did not differ from healthy woman.•Birth weights between statin exposed and non-exposed pregnancies was similar.•No severe skeletal abnormalities or developmental delay occurred in statin exposed fetusses.