Both, somatic and germline (heterozygous and biallelic) variants in SUFU were found to be associated with cerebellar medulloblastoma.25 A heterozygous truncating germline variant in SUFU was observed in familial meningioma.26 Given the fact that haploinsufficiency caused by truncating loss-of-function variants throughout SUFU is the underlying molecular mechanism in both COMA and BCNS, additional genetic and/or nongenetic modifiers must exist that drive the phenotypic expression toward a specific clinical entity. This evidence concerns the gene SUFU and nevoid basal cell carcinoma syndrome.