Neuromuscular disease (NMD) is a heterogeneous disease that can affect the peripheral nervous system including motor neurons, sensory neurons, muscles and neuromuscular junctions, seriously affecting the quality of life of patients, and its outcome is irreversible. Early diagnosis and treatment are critical to the formulation of NMD treatment strategies and the guarantee of quality of life of patients. In the past, the diagnosis of NMD mainly relied on the determination of creatine kinase, electromyography, the measurement of nerve conduction velocity, genetic testing, and muscle biopsy. However, these methods are not effective or invasive or affected by gene variation. In recent years, skeletal muscle ultrasound imaging has been increasingly used as a useful supplement to clinical and electrophysiological examination of NMD, but the quantitative evaluation of skeletal muscle structure and function needs to be detailed. This article reviews the current research status of quantitative ultrasound in the evaluation of skeletal muscle structure and function of NMD.