Marshall-Smith syndrome is a rare clinical disorder characterized by accelerated bone maturation, dysmorphic facial features, airway abnormalities and death in early infancy because of respiratory complications. Although patients with Marshall-Smith syndrome have several features with potential anaesthetic problems, previous reports about anaesthetic management of these patients do not exist. We present a case, in which severe hypoxia developed rapidly after routine anaesthesia induction in an eight-month-old male infant with this syndrome. After several unsuccessful attempts the airway was finally secured by blind oral intubation. After 2 weeks, laryngeal anatomy was examined with fibreoptic laryngoscopy which revealed significant laryngomalacia. Laryngoscopy was performed without problems with ketamine anaesthesia and spontaneous breathing. The possibility of a compromised airway should always be borne in mind when anaesthetizing patients with Marshall-Smith syndrome. Anaesthesia maintaining spontaneous breathing is safest for children with this syndrome. If tracheal intubation or muscle relaxation is required, precautions are needed to maintain a patent airway. Muscle relaxants should possibly be avoided before intubation.