Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by progressive impairments in behavior, executive function, and language, primarily affecting individuals under the age of 65. This disorder is associated with expressive and receptive anomia, word comprehension deficits, and behavioral symptoms such as apathy, loss of empathy, and disinhibition, all of which closely correlate with functional impairment in daily activities. Despite substantial efforts, research on occupational therapy (OT) interventions has yet to demonstrate clear benefits in managing the disease. The aim of this study is to investigate OT interventions and assess their efficacy, with a specific focus on individuals suffering from FTD. We systematically conducted searches on two databases, namely Medline and Science Direct, spanning a ten-year period from 2003 to 2023, in accordance with the PRISMA guidelines. Eleven studies met the inclusion criteria. OT interventions targeted both patients and caregivers and yielded significant positive improvements in their lives. A key focus of these interventions was to teach acceptable alternatives to the behaviors exhibited by FTD patients, as these behaviors are strongly influenced by the disease itself. OT contributes positively to enhancing the quality of life of FTD patients and alleviating the caregiving burden experienced by those providing long-term care to these patients.