We present our experience with 16 children with Moyamoya disease/variant diagnosed at the Royal Alexandra Hospital for Children, Westmead, Sydney, Australia in the period between January 1982 and March 2004. Thirteen of these patients had one of the different revascularisation procedures. We reviewed the modes of presentation and the long-term outcome in these children. In our series the functional outcome was related to the functional status at presentation and was not related to the type of surgical procedure or age at presentation. We believe this is the largest reported series of Moyamoya disease/variant in Australia.