Purpose Intellectual disability (ID) is prevalent in 1 per cent of the population. Premenstrual syndrome (PMS) affects up to 5 per cent of the general population of adult women. Identification of PMS is challenging in women with ID due to differences in communication. Management of PMS in the ID population requires careful consideration of baseline function, co-existing mental and physical health problems, drug interactions as well as complex ethical considerations. The paper aims to discuss this issue. Design/methodology/approach Prospero-registered systematic review (CRD42019119398) of papers exploring the diagnosis and management of patients with PMS and ID (n=414). In total, 35 relevant titles were identified and 27 full text papers were assessed for eligibility, resulting in 10 studies for final qualitative analysis. Findings Ten original research papers were included. There are no standardised symptom criteria for diagnosis of PMS in women with ID. Studies relied on observer-reported data. All papers demonstrated higher rates of PMS in women with ID compared with the general adult population. Management was not standardised and varied between centres. Mainstays of treatment included non-steroidal anti-inflammatories, combined oral contraceptive pills and intramuscular progesterone. Newer evidence suggests levonorgestrel intrauterine systems may be appropriate. There was no quantitative method of establishing success of management. Practical implications A modified symptom diary should be used for diagnosis in this population. Differentiation between cyclical behavioural change due to pain vs mood disturbance remains challenging. Conservative, psychological and medical management should be the mainstay of treatment, with surgery considered in exceptional cases. Originality/value This paper demonstrates the current limited evidence for the management of PMS in women with a diagnosis of ID and offers an overview of the current options for managing these patients’ symptoms.