We present a case series of two siblings from a consanguineous family with genetically confirmed POLR3B-related hypomyelinating leukodystrophy type 8 (4H syndrome), a rare autosomal recessive disorder characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism. Here, POLR3B is linked to hypogonadotropic hypogonadism.