Costello syndrome is a rare genetic disorder characterised by growth and mental retardation, macrocephaly, short neck and macroglossia. Cardiac involvement can also occur in Costello syndrome and is presented in the form of hypertrophic cardiomyopathy, tachyarrythmias and valvular dysfunction. Nervous system involvement including ventriculomegaly, hydrocephaly and Chiari type(1) malformation are also common. Predisposition of papillomata and malignant tumours are high. General anaesthesia practice in patients with Costello syndrome may be complicated by difficult airway because of macrocephaly, short neck, macroglossia and oral or laryngeal papillomas. The airway management and cardiac abnormalities are the major concerns of an anaesthesiologist in Costello syndrome. We report the anaesthetic management of ventriculo-peritoneal shunt replacement for hydrocephaly in an 18-month-old child with Costello syndrome.