Revisiting after time their clinical phenotype, thesuggestive matching with the autosomal dominant syndrome Poikiloderma,Hereditary Fibrosing with Tendon Contracture, Myopathy and Pulmonary fibrosis(POIKTMP) was confirmed by identification of the c.1879A>G (p.Arg627Gly)alteration in FAM111B. We compare the overall clinical signs ofour patients with those of reported carriers of the same mutation and presentthe up-to-date mutational repertoire of FAM111B and the relatedphenotypic spectrum. The gene discussed is FAM111B; the disease is pulmonary fibrosis.