Dandy Walker Syndrome is rare congenital malformation of the brain occurring in one in every 30,000 births.It is characterized by a classical neuropathological triad consisting of hypoplasia of the cerebellar vermis, cystic dilatation of the fourth ventricle and hydrocephalus.We report a 7 year female child diagnosed as Dandy Walker Syndrome at four months of age, presented with excessive crying, irritability, delayed milestones and increase in the size of the head. She was operated at 6 months of age for VP shunt surgery, as the symptoms recurred; she was posted for revision of VP shunt surgery in our institution.As the case was anticipated as a difficult airway due to the large size of the head and inadequate neck extension which made airway management challenging in this patient.Hence, careful evaluation of airway anatomy, appropriate positioning and airway management intra operatively were essential to anaesthetize a patient afflicted with Dandy Walker Syndrome.A rare syndrome posing considerable airway challenge, managed successfully.
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