The trait is Autosomal recessive. Primary ciliary dyskinesia is the major patho-physiology with Sinusitis, Bronchiectasis and Situs Invertsus as the characteristic features. Preoperatively Respiratory and Cardiovascular system require special optimisation because of the presence of ciliary dysfunction, the management should be customised to avoid problems in the perioperative period. In present case few pre-emptive efforts like avoiding high FiO2, anticholinergics and thorough endobronchial suctioning utilizing fiberoptic bronchoscope before extubation helped us to avoid anticipated complications. Features important from anaesthetic view point include bronchiectasis and pan sinusitis causing airwayobstruction, gas trapping and arterial hypoxemia that can later proceedto acute respiratory failure.