WHIM syndrome (warts, hypogammaglobulinemia, infections, myelokathexis) is a rare multi-system combined immunodeficiency most often caused by autosomal dominant pathogenic variants in the CXCR4 gene region coding for the C-terminus of the C-X-C chemokine receptor type 4. Here, CXCR4 is linked to agammaglobulinemia.