Takayasu disease is a chronic, progressive inflammatory vasculitis of large and medium-sized vessels, which commonly presents in adulthood. This case report describes a 2 year old girl who presented with acute heart failure and complete occlusion of the aortic arch and was subsequently diagnosed with Takayasu disease. As far as we can determine, this is the first report of such an acute and atypical presentation of Takayasu disease at such a young age.