Patients with frontotemporal dementia (FTD) often present with an asymmetric left or right-sided anterior cerebral perfusion abnormality that is associated with differential behavioral symptoms. However, whether patients with primarily right versus left FTD also have unique neuropsychological characteristics has not been previously investigated. Comparisons of 11 patients with right-sided FTD and 11 with left FTD indicated that the 2 patient groups showed relatively distinct cognitive profiles. Patients with right FTD exhibited relatively worse performance on PIQ than VIQ, and on select nonverbal executive tasks relative to their verbal analogs (e.g., design fluency < word generation; Picture Arrangement < word sequencing). In contrast, patients with left FTD showed the opposite pattern. In addition, the 2 patient groups differed on several absolute test scores; patients with right FTD demonstrated more errors and perseverative responses, and worse percent conceptual level responses, on the Wisconsin Card Sorting Test, while the left FTD patients obtained significantly worse scores on the Boston Naming Test, and Stroop word reading and color naming. Verbal and nonverbal memory, mental speed, visual perceptual-constructional skill, and IQ subtest scaled scores did not significantly differ between groups. These data indicate that FTD should not be viewed as a unitary disorder, and that neuropsychological testing holds promise for the differential diagnosis of right versus left FTD.