Laryngeal (laryngotracheo-oesophageal) clefts are relatively rare, but are being reported with increasing frequency. The condition is of particular interest to paediatric anaesthetists, but may present in adults and may be encountered by any anaesthetist called to assist an infant in respiratory distress. Its presentation has features in common with most respiratory disorders of infancy and it should be considered in the differential diagnosis. Increased mortality and morbidity is linked to failed early diagnosis. Successful management requires both an awareness of the possible diagnosis and employment of modified techniques to avoid potential pitfalls of intubation. Perioperative management presents anaesthetic challenges at all stages. This article aims to discuss these challenges and highlight the associated syndromes in which the defect may be encountered.