The purpose of this study was to assess the convergent and discriminative validity of the Montreal Cognitive Assessment (MoCA) as a screening tool for cognitive dysfunction in Huntington's disease (HD). Twenty HD patients with cognitive deficit and 23 normal controls (NC) without cognitive deficit were matched for age, sex, and education. The mean MoCA score was 20.5 (SD = 5.5) in HD and 27.5 (SD = 2.2) in NC. The MoCA correlated in both samples with the brief cognitive battery composite score (r = .81, p < .001). With the screening and diagnostic cutoff scores determined at <26 points, the MoCA showed a sensitivity of 94% and a specificity of 84% in the detection of cognitive dysfunction in HD. The area under the receiver-operating characteristics curve (95% confidence interval) for the MoCA was 0.90 (0.809-0.997), p < .001. Our results show that the MoCA is a suitable tool for assessing cognitive dysfunction in patients with HD.