Mutations in the USB1 (C16orf57) (U6 snRNA biogenesis 1, OMIM #613276) gene give rise to autosomal‐recessive Clericuzio‐type poikiloderma with neutropenia (PN, OMIM #604173), characterized by early‐onset poikiloderma (appearing in the first year of life), nail abnormalities, palmoplantar hyperkeratosis, skeletal defects as well as noncycling neutropenia and recurrent sinopulmonary infections (Colombo et al. Here, USB1 is linked to neutropenia.