Background/Aims: Although most patients with frontotemporal dementia (FTD) present with neuropsychiatric symptoms, the frequency of psychotic symptoms is unclear. This study aims to determine the prevalence of psychotic symptoms in a large cohort of well-diagnosed and followed FTD patients compared to age-matched patients with Alzheimer’s disease (AD) and to further review the literature on psychosis in FTD. Methods: Delusions, hallucinations and paranoia were evaluated among 86 patients who met consensus criteria for FTD, had frontotemporal changes on functional neuroimaging and were followed for 2 years. They were compared to 23 patients with early-onset AD on a caregiver-administered psychiatric questionnaire. Results: Among the FTD patients, only 2 (2.3%) had delusions, 1 of whom had paranoid ideation; no FTD patient had hallucinations. This was significantly less than the AD patients, 4 (17.4%) of whom had delusions and paranoia. Other investigations fail to establish a significant association of psychosis with FTD. Conclusions: These findings, and a literature review, indicate that psychotic symptoms are rare in FTD, possibly due to limited temporal-limbic involvement in this disorder.