In approximately 5% of cases of Huntington’s disease, onset is before 20 years of age, and these cases are defined as juvenile Huntington’s disease (JHD). The clinical and pathologic features of JHD overlap with those of typical adult-onset disease, so it is not a distinct entity. As a generalization, however, dystonia and bradykinesia are more likely to occur earlier in the disease process, epilepsy is more frequent, the average CAG repeat length is longer, and the pathology within the brain is more widespread. There are conflicting reports in the literature regarding both disease duration and disease progression. Management of JHD patients, and their families, requires a multidisciplinary, symptomatic, and supportive approach. Despite the lack of an evidence base for pharmacologic management and other interventions, helpful reviews and suggestions are available.