A 12-year-old child with terminal ischemic heart disease as a result of homozygotous familial type IIa hypercholesterolemia received an orthotopic heart transplant and, 21 days later, an orthotopic liver transplant. Six months after heart transplantation, the patient is asymptomatic and evidences normal liver function and cholesterol levels; there are no signs of heart rejection. To our knowledge, this is the first instance of two-step heart-liver transplantation with organs from different donors.