Degeneration of the striatum is a key component of Huntington’s disease, yet the role of other brain regions in the disease process is virtually unexplored. Recent research has discovered novel anatomical connections between the cerebellum and striatum. Our study measures volume, function and development of the cerebellum in a unique sample of children with juvenile-onset Huntington’s disease. Identifying the role of the cerebello-striatal circuit in Huntington’s disease may lead to novel and targeted therapeutic interventions.