Background and purpose: Huntington disease (HD) is a progressive neurodegenerative disorder. There are no HD-specific measures to assess for end-of-life (EOL) preferences that have been validated for clinical use. The purpose of this study is to demonstrate reliability and validity of three HD-specific EOL measures for use in and clinical research settings. Methods: We examined internal reliability, test–retest reliability, floor and ceiling effects, convergent and discriminant validity, known groups’ validity, measurement error, and change over time to systematically examine reliability and validity of the HDQLIFE EOL measures. Results: Internal consistency and test–retest reliability were > 0.70. The measures were generally free of floor and ceiling effects and measurement error was minimal. Convergent and discriminant validity were consistent with well-known constructs in the field. Hypotheses for known groups validity were partially supported (there were generally group differences for the EOL planning measures, but not for meaning and purpose or concern with death and dying). Measurement error was acceptable and there were minimal changes over time across the EOL measures. Conclusions: Results support the clinical utility of the HDQLIFE EOL measures in persons with HD. © 2019, Springer-Verlag GmbH Germany, part of Springer Nature.