Background: Frontotemporal dementia (FTD) is one of the most common types of dementia in persons younger than 65 years of age. Diagnosis is often delayed due to slow, gradual decline and misinterpretation of ‘non-typical’ dementia symptoms. Informal carers of people with FTD experience greater levels of overall burden than carers of people with other forms of dementia. Objectives: The aim of this systematic review was to describe the subjective experience of being an informal carer of a person with FTD and to identify the specific needs, coping strategies and helpful support resources of this carer population. Methods: Four electronic databases were used to search for published literature presenting experiences of carers of people with FTD between January 2003 and July 2019. Search strategy followed PRISMA guidelines. Findings were analysed using framework analysis, employing five stages of analysis to develop a coding index and thematic framework that included key aspects of the carer experience, which were grouped into themes and presented in a narrative format. Results: 1213 articles were identified in total. Twelve studies were included in the final synthesis of the review. Six themes were identified: ‘Challenging road to and receipt of diagnosis’, ‘relationship change and loss’, ‘challenging experiences in caring’, ‘positive experiences and resilience’, ‘coping’ and ‘support needs’. Discussion: Findings highlight an increased need for carers of people with FTD to receive support during the pre-diagnostic stage, including support to manage symptoms. Further research should explore relationship changes and loss amongst carers to inform approaches for carer support. In conclusion, the lack of knowledge and unique needs of carers highlight the importance of public awareness campaigns and healthcare professional education to support carers with FTD symptom impact.