Huntington’s disease (HD) is a complex, hereditary, and life-limiting neurodegenerative condition. It brings a range of symptoms and difficulties that are often profoundly debilitating. This includes physical symptoms (e.g., chorea and dysphagia), cognitive difficulties (e.g., executive function impairment, disorientation, and apathy), and emotional changes (e.g., low mood, anxiety, irritability, and self-harm). These challenges significantly impair quality of life and independence over time, with the person eventually requiring total support in later stages. Family caregivers are also impacted by HD, often experiencing high levels of psychological distress which may include low mood, anxiety, and cognitive challenges. Despite the psychosocial burden of living with HD for individuals and families, psychological interventions and services tailored to their needs are rare. Acceptance and commitment therapy (ACT) may comprise a route to appropriately specialized support; this approach uses mindfulness and behavioral change to help individuals live with and manage difficult emotions and circumstances, undertaking committed action aligned with their personal values. Evidence across a range of conditions demonstrates the effectiveness of ACT, including for neurological disorders. ACT may be particularly helpful for those affected by HD, as HD is progressive and currently incurable, meaning that distress is often ongoing and requires compassionate and flexible coping strategies. Thus, ACT offers a promising framework for improving psychological well-being for individuals affected by HD. Further research is needed to evaluate the feasibility and effectiveness of ACT-based interventions for the HD community and to address the current gap in psychological evidence-based care for those affected by HD.