In conclusion, a dramatic decrement in high-frequency RNS, especially in the trapezius, was found in three patients with GFPT1-related CMS from southwestern China, which has never been reported, indicating that the trapezius may be a suitable targeted muscle for performing RNS in patients under the consideration of GFPT1-related CMS. Here, GFPT1 is linked to congenital myasthenic syndrome.