The most common Sicilian founder mutation named BRCA1–5083del19 (HGVS nomenclature: c.4964_4982del; p.Ser1655fs) (26, 52, 53), usually detected with high frequency in women with HBOC syndrome, was not found in the examined male population affected by HBOC syndrome-associated tumours. The gene discussed is BRCA1; the disease is neoplasm.