CYLD cutaneous syndrome (CCS), also called Brooke-Spiegler syndrome, is characterized by the development of multiple adnexal skin tumors that can include cylindromas, spiradenomas, and trichoepitheliomas.1, 2, 3 Genetic linkage studies suggest that CCS is a single-locus disease,4 with exonic germline pathogenic CYLD variants shown in up to 85% of cases.5 Here, CYLD is linked to skin neoplasm.