Since the last comprehensive clinical care guideline for FSHD was published in 2015, advances in research, therapy development, and patient advocacy have motivated the need for an updated guideline. Importantly, several novel therapies are in clinical trials with the prospect of new treatments coming on the market in the next few years, raising the urgency of diagnosing and optimising patient care as part of trial and treatment readiness. In 2021-2022, the FSHD Care Considerations Working Group was formed with 40 clinicians from a wide range of disciplines and diverse nations to develop recommendations aimed at improving care standards, health outcomes, and quality of life for people with FSHD. Individuals with FSHD provided input at all phases. The working group identified 13 topics to include in this update, 9 of which were addressed in the 2015 guideline. The 4 new topics are communication, speech, and swallow; mental health; pregnancy, birth, and parenting; and emerging pharmacologic therapies. The recommendations have evolved significantly from past guidelines, driven by advances in research, input by individuals with FSHD, and the prospect of gene-targeting therapies coming on the market in the near future. Notably, genetic testing is now recommended for all individuals with clinical signs, with or without a family history of FSHD. Randomised controlled trials indicate that light to moderate aerobic exercise, resistance training, high-intensity interval training, cognitive behavioural therapy, and acceptance and commitment therapy may be beneficial not only for muscle function and fitness but also pain, chronic fatigue, and mental health. This guideline also established consensus recommendations based on the cumulative experience of specialist clinicians who have managed many FSHD patients in diverse geographic regions and medical practice settings.