AbstractObjectiveThe mainstay management of hyperphagia and obesity in Prader–Willi syndrome (PWS) relies on dietary restrictions, strict supervision and behavioural modifications, which can be stressful for the patient and caregiver. There is no established pharmacological strategy to manage this aspect of PWS. Theoretically, glucagon‐like peptide‐1 (GLP‐1) receptor agonists (GLP1‐RA) used in patients with obesity and type 2 diabetes mellitus (T2DM) may be efficacious in weight and glycaemic control of PWS patients. We conducted a systematic review of the literature to summarize the evidence on the use of GLP1‐RA in PWS patients.DesignPrimary studies were searched in major databases using key concepts ‘Prader–Willi syndrome’ and ‘GLP1 receptor agonist’ and outcomes, ‘weight control OR glycaemic control OR appetite regulation’.ResultsTen studies included, summarizing GLP1‐RA use in 23 PWS patients (age, 13–37 years), who had used either exenatide (n = 14) or liraglutide (n = 9) over a duration of 14 weeks to 4 years. Sixteen (70%) of these patients had T2DM. Ten patients experienced improvement in body mass index, ranging from 1.5 to 16.0 kg/m2, while improvement in HbA1c was seen in 19 of 23 cases, ranging between 0.3% and 7.5%. All five studies reporting appetite or satiety showed improvement in satiety levels. There were no reported serious side effects.ConclusionsGLP1‐RA appears safe in PWS patients and may have potential benefits for weight, glycaemic and appetite control. Nonetheless, we also highlight a significant gap in the literature on the lack of well‐designed studies in this area, which limits the recommendation of GLP1‐RA use in PWS patients at present.