A 5-month-old boy required sedation after a cleft lip repair. He was sedated with propofol and intermittent fentanyl, requiring escalating doses over the subsequent 48 h. On the second post-operative day he developed a metabolic acidosis followed by multiple cardiac dysrhythmias, hepatic and renal failure. Propofol was stopped. His multisystem organ failure gradually resolved after initiation of charcoal haemoperfusion. Further investigation demonstrated an abnormality in acylcarnitine metabolism, similar to that found in one previous case report.