The patient (P-08) with a pathogenic CXCR4 mutation (NM_003467.2:c.966_967del, p.(R326fs)) showed permanent neutropenia, several neutropenic fever events with or without infections, inguinal hernia, incomplete cleft lip and serum IgG and IgA deficiencies. Here, CXCR4 is linked to Inguinal hernia.