BACKGROUND: The neuroanatomic basis of affective processing deficits in Huntington disease is insufficiently understood. We investigated whether Huntington disease-related deficits in emotion recognition and experience are associated with specific changes in grey matter volume. METHOD: We assessed grey matter volume in symptomatic patients with Huntington disease and healthy controls using voxel-based morphometry, and we correlated regional grey matter volume with participants' affective ratings. RESULTS: We enrolled 18 patients with Huntington disease and 18 healthy controls in our study. Patients with Huntington disease showed normal affective experience but impaired recognition of negative emotions (disgust, anger, sadness). The patients perceived the emotions as less intense and made more classification errors than controls. These deficits were correlated with regional atrophy in emotion-relevant areas (insula, orbitofrontal cortex) and in memory-relevant areas (dorsolateral prefrontal cortex, hippocampus). LIMITATIONS: Our study was limited by the small sample size and the resulting modest statistical power relative to the number of tests. CONCLUSION: Our study sheds new light on the importance of a cognitive-affective brain circuit involved in the affect recognition impairment in patients with Huntington disease.