Introduction and objectives Exogenous lipoid pneumonia (ELP) is a lung inflammatory disease with low prevalence and has the feature of external lipid substances presented in the alveoli. Therapeutic lung lavage (segmental bronchoalveolar lavage and whole lung lavage) has been gradually recognized as an important therapy for the disease. There was no comprehensive summary on its efficacy and safety. Methods We searched PubMed, Embase, Cochrane Library, CNKI, Wanfang Database, clinicaltrials.gov, and the references of included studies. After study selection, data extraction and quality assessment, we performed a qualitative description of current data. Results We included 90 ELP patients from 25 case reports and 8 case series studies. Eighty-four (93.3%) patients received segmental bronchoalveolar lavage and six (6.7%) patients received whole lung lavage. Eighty-seven (96.7%) patients got clinical improvement after lavages, while three (3.3%) patients had no improvement and eventually died. The follow-up status was reported in 29 patients, of whom 24 patients remained well without any use of drugs and 4 patients remained well with some periods of corticosteroids. One patient endured recurrence. The radiological change was reported in 72 patients, of whom 41 (56.9%) patients had full resolution until the last follow-up. Two studies reported acute pulmonary edema and transient hypoxemia during lavages. Conclusions Therapeutic lung lavage might be an effective and safe therapy with long-term benefits for ELP. Current studies were all case reports and case series with relatively high risk of bias. Prospective controlled studies are needed to explore the actual efficacy, safety, individualized indications, and optimized treatment procedures of therapeutic lung lavage for ELP.