Children who have Dandy-Walker syndrome present a particular set of challenges to their teachers as do any children with special needs. Children affected with this particular syndrome can be especially challenging for teachers because this syndrome is relatively rare and so teachers are highly unlikely to have had the chance to work with many students with the syndrome. The fact that the syndrome present itself with a wide range of symptoms also makes it challenging for schools to derive appropriate Individual Educational Plans for students with Dandy-Walker syndrome, as does the fact that many children with Dandy-Walker syndrome have a number of other, often serious, cognitive and physical disabilities. This paper examines this syndrome and looks at how teachers can create the most positive, least restrictive environment for students with Dandy-Walker Syndrome.