Interestingly, germline heterozygous truncating and loss-of-function variants in SUFU (OMIM *607035) were observed in the basal cell nevus syndrome (BCNS, Gorlin–Goltz syndrome, OMIM 109400), a cancer-predisposing condition with variable developmental and skeletal anomalies.22,23 BCNS may also be associated with a heterozygous germline pathogenic variant in PTCH1. BCNS is mainly characterized by lamellar calcification of the falx, jaw keratocysts, palmar/plantar pits, and multiple basal cell carcinomas as major diagnostic criteria. Here, SUFU is linked to basal cell carcinoma.