Huntington's disease (HD) is a genetic brain disease characterized by loss of capacity in movement control, cognition, and emotional regulation over a period of about 30 years. Since it is well established that clinical impairments and brain atrophy can be detected decades prior to receiving a clinical diagnosis, functional neuroimaging efforts have gained momentum in HD research. In most brain disorders, there is accumulating evidence that the clinical manifestations of disease do not simply depend on the extent of tissue loss, but represent a complex balance among neuronal dysfunction, tissue repair, and circuitry reorganization. Based upon this premise, functional neuroimaging modalities may be more sensitive to the earliest changes in HD than are structural imaging approaches. For this review, PET and fMRI studies conducted in HD samples were summarized. Strengths and limitations of the utilization of functional imaging in HD are discussed and recommendations are offered to facilitate future research endeavors.