Facioscapulohumeral muscular dystrophy (FSHD) is a common muscular disorder. At present, treatments for FSHD have limited effects on the muscle function of patients. A famous Chinese medicine formula, Buzhong Yiqi (BZYQ), has shown promising effects on several muscular diseases, but evidence regarding its effect on FSHD is lacking. This study aimed to examine the effect of BZYQ on FSHD. A 15-year-old girl suffered from progressive muscle weakness, with a genetically confirmed diagnosis of FSHD. Except for routine FSHD management, the patient received BZQY every day. The muscle strength of the patient remarkably increased after discharge. This study was novel in reporting a significant improvement in muscle function in a patient with FSHD treated with an integrated approach of BZYQ and routine management. Therefore, BZYQ might be a potential treatment for FSHD, requiring further investigations. Copyright © 2020 The Author(s). Published by Elsevier Inc. All rights reserved.