Objective: To evaluate the prrioperative airway function and breathing pattern after cleft palate repair at an early age in infants and children with Pierre Robin sequence.Patients and Methods: Six consecutive patients (4 boys and 2 girls) with Pierre Robin sequence underwent primary cleft palate repair using the won Langenbeck technique from May 2001 to February 2004. Pre- and postoperative polysomnographic studies were performed and prrioperative arterial oxygen saturation was monitored.Results: All the patients experienced some degree of hypoxaemia during the endotracheal intubation procedure. Only 1 patient had hypoxaemia in the postanaesthetic recovery period. No obvious difference was found in the apnoea and hypopnoea index among patients when comparing preoperative and postoperative polysomnographic findings.Conclusions: Hypoxaemia was likely to occur during intubation in infants and children with Pierre Robin sequence, while a pre-existing disordered breathing pattern, especially if severe, predisposed the patients to postoperative hypoxaemia. The need for an experienced specialist anaesthetist and the need for postoperative intensive care are emphasised.