The estimated age-standardized incidence rate is highest in Western and Northern Europe, North America, Australia and New Zealand.1 In Slovenia, PCa is the most common solid neoplasm in men (excluding skin cancer) with a share of 18.6% and an estimated incidence rate of 162 per 100,000 in 2022.2 The incidence rate of PC in Western countries and Slovenia has increased dramatically from the early 1990s to the last decade, mainly due to the use of prostate specific antigen (PSA) as a tumor marker for PCa.2,3,4. The gene discussed is KLK3; the disease is pachyonychia congenita.