BACKGROUND: The purpose of this descriptive, retrospective analysis was to develop a demographic and clinical profile of nursing home residents with a diagnosis of Huntington's disease (HD). METHODS: Queries were made of a large data repository of linked and de-identified Minimum Data Set version 3.0 and prescription claims records, for the time period of October 1, 2010 through March 31, 2012. RESULTS: Of 249,811 residents, 340 (0.14%) had a diagnosis of HD; 61% were female and 77.9% were Caucasian. The age range mode was 55-59 years (15%). Approximately one-half of the residents with HD exhibited communication difficulties, 78% had moderate or severe cognitive impairment, and most have significant functional limitations. Depression, dementia, anxiety, psychosis, and bipolar disease were present in 59.4%, 50.9%, 35.9%, and 23.2%, and 9.7%, respectively. Only 21% of residents with HD exhibited troublesome behavioral symptoms. Comorbidities of diabetes and cancer were uncommon (0.3%). Use of physical restraints (excluding bed rails) was considerably higher in residents with HD than in the general nursing home population. Falls were documented in almost one-half of residents. Antipsychotics were used in 61.6% of residents; 16.2% had psychotic symptoms. One was treated with tetrabenazine. Anxiolytics were received by 59.1% of residents, whereas only 35.9% had anxiety noted on Minimum Data Set records. CONCLUSIONS: The prevalence of HD in US nursing homes is very low (0.14%). Affected residents have significant cognitive and functional impairments, but problematic behaviors are present in only a minority. Serious comorbidities such as cancer and diabetes are rare. Antipsychotics, antidepressants, and anxiolytics are the mainstays of treatment.