Background Lymphangioleiomyomatosis (LAM) is associated with progressive dyspnoea and exercise intolerance, but despite the central role of physiotherapy on pulmonary rehabilitation, there is a huge lack of physiotherapy approaches used specifically for LAM patients. Objective to identify the physiotherapeutic strategies used in the treatment of patients with LAM. Materials and methods This is a systematic review of literature. Searches were performed (in PubMed, Lilacs, Embase and PEDro databases) with the keywords “Lymphangioleiomyomatosis” and “Physiotherapy,” and its variations. Articles describing physiotherapy interventions were included in the study. Data extracted from the studies were authors, year, country of publication, sample size, physiotherapy intervention, time/frequency/duration of intervention protocols, instruments used to measure results and main findings. Methodological quality of studies was evaluated by PEDro Scale (clinical trials), Newcastle–Ottawa Scale (NOS; observational studies) and CARE checklist (case reports), respectively. Results A total of 82 articles identified, three duplicates were removed, 71 studies were excluded after title and abstract reading and four after full-text reading, all due to absence of association with the study topic. Four studies were included in the present review. Cardiorespiratory physiotherapy with endurance and resistance training were identified as physiotherapeutic strategies to improve lung function, functional capacity, depression symptoms and quality of life in LAM. Conclusions Endurance and resistance training is the keystone for physiotherapy in patients with LAM, but despite the reported benefits, there is a huge lack of studies related to the modalities, safety and dosage of physiotherapy prescription for patients with LAM. KEY MESSAGES Lymphangioleiomyomatosis (LAM), a rare disease, leads to progressive dyspnoea and exercise intolerance; Physiotherapy can improve dyspnoea and exercise intolerance in LAM through endurance and resistance exercises.