Further evidence came from Passos et al who described a patient with FAP from a known pathogenic APC germline variant who underwent resection of an ectopic craniopharyngioma in the cerebellopontine angle.8 In addition to the germline mutation, the tumor carried a second somatic variant affecting the other allele, leading to a premature stop codon, and subsequently biallelic loss of APC. Here, FAP is linked to craniopharyngioma.