Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron diseases. Until now, it lacks effective drugs for its treatment, and the median survival time of ALS is reported as only 20 to 48 months after the onset of symptoms. Acupuncture served as part of traditional Chinese therapy, has been widely applied to clinical practice for patients with ALS but lacks studies to verify its efficacy. This study provides a protocol of systematic review, with which we will comprehensively verify the effects of acupuncture on ALS with evidence-based studies.The eligible studies will be collected from 4 English databases (the MEDLINE via PubMed, the Cochrane Library, EMBASE, the Web of Science, and Ovid database), and 4 Chinese databases (China Science and Technology Journal Database, Chinese Biomedical Literature Database, Wan-fang Database, China National Knowledge Infrastructure) from October 2000 to October 2022. The primary outcome measure is the change in amyotrophic lateral sclerosis functional rating scale-revised (ALSFRS-R) scores. We will use RevMan V.5.3 software to calculate the data synthesis and will conduct meta-analysis based on the collected data.The primary outcome measure is the change in ALSFRS-R scores, and secondary outcome measures included changes in forced vital capacity, grasping power, pinch strength, modified Norris Scale, ALS assessment questionnaire-40, and time to activity of daily living-independent will be measured and comprehensively assessed to evaluate the effect of acupuncture on ALS from this systematic review and meta-analysis.The systematic review and meta-analysis will assess the effect of acupuncture in the treatment of ALS with up-to-date clinical evidence.PROSPERO CRD 42019124785.