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A boy in early childhood with genetically confirmed familial hypercholesterolaemia was found to have an unusually low low-density lipoprotein cholesterol level. He had undergone a distal ileal resection and right hemicolectomy for ileal atresia and microcolon in the neonatal period. This case serves as a reminder of the surgical interventions sometimes used to treat severe hypercholesterolaemia in the pre-statin era.