To date, the majority of studies have examined the prevalence of TP53 germline mutations among families identified through the genetics clinics and these studies have shown that the majority of families with germline TP53 mutations fulfill either the original Li-Fraumeni syndrome (LFS) criteria, Li-Fraumeni-like (LFL) criteria or the Chompret criteria and are present with a family history of at least one of the four LFS-linked cancers (breast, bone or soft tissue sarcomas, brain tumors and ACC) [1,5,8,9]. The gene discussed is TP53; the disease is lamellar ichthyosis.