Anaesthetizing patients with Long QT Syndrome is a major challenge, as the potential for sudden catastrophic cardiovascular collapse is well known. We present a 15-year-old boy with Long QT Syndrome who presented for an elective renal transplant. All electrolyte concentration abnormalities were corrected preoperative and adequate beta-blockade was maintained. The patient was given a target controlled infusion of propofol, together with opioids and atracurium. Anaesthesia was uneventful and the patient was extubated at the end of the surgical procedure.