The identification of a predisposition syndrome for medulloblastoma caused by a germline PV in SUFU or PTCH1 gene is fundamental, because the presence of an underlying genetic anomaly can have an impact on the therapeutic management, due to the different behavior of SUFU or PTCH1-related medulloblastomas, including differences in their responses to SHH inhibitors [22]. The gene discussed is SHH; the disease is medulloblastoma.