Collectively, this report consolidates the disease‐causing role of biallelic variants in GGPS1, demonstrates that hearing loss and ovarian insufficiency might be variable features of the GGPS1‐associated muscular dystrophy and implicates impaired substrate and co‐factor binding as potential pathogenic mechanisms in this novel form of congenital muscular dystrophy. Here, GGPS1 is linked to muscular dystrophy.