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. 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 optimizing 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. A systematic review of published evidence was carried out. For topics where evidence from randomized controlled trials is lacking, a consensus process was used to develop care recommendations. Here we will present an overview and key recommendations from the updated guidelines, including the latest on genetic testing, rehabilitation, pain and fatigue, and topics not covered in the previous guideline, including communication, speech, and swallow; mental health; pregnancy, birth, and parenting; and new therapies.