Among the causes of death, acute cerebrovascular accidents (6/12; 50% of patients) (patients No 1, 2, 4, 9, 19, and one child without any pathogenic variants in SMARCAL1) and sepsis due to immunodeficiency (3/12; 25% of patients) (patients No 8, 14, and 18) were the most frequent; two children (17%) died due to uncontrolled oedema and hydrothorax (patients No 5 and 6), and there were no data on one child’s (8%) cause of death (patient No 3). Here, SMARCAL1 is linked to Sepsis.