FILS syndrome (facial dysmorphism, immunodeficiency, livedo, and short stature) is a recently described condition caused by variants in POLE1, encoding the catalytic subunit of polymerase E. We describe the second family with a homozygous variant in POLE1, and a more severely affected individual, suggesting a broader phenotypic spectrum for this condition. The gene discussed is POLE; the disease is Immunodeficiency.