Gomez-Lopez-Hernandez syndrome (GLHS) is an infrequent condition first reported by Manuel Gomez in 1979 in a 5-year-old Caucasian girl (Gomez 1979). He presumed that the patient had focal dermal and cerebello-trigeminal dysplasia due to an interruption in ectodermal development, which is responsible for the formation of the trigeminal placodes, overlay epidermis, alar plate of the rhombencephalon, and motor nucleus of cranial nerve V (Gomez 1979). Similarly, in 1982, Lopez-Hernandez reported partial scalp alopecia, ataxia, intellectual disability, and trigeminal anesthesia, combined with rhombencephalosynapsis (RSE), in two Mexican girls (López-Hernández 1982). This syndrome is clinically characterized by a neurocutaneous triad of RSE, partial scalp alopecia, and trigeminal anesthesia, with trigeminal anesthesia present in 50–60% of cases (Choudhary et al. 2021) (Figs. 1 and 2).