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X-linked hyper-IgM syndrome (XHIGM) is a congenital immunodeficiency in which the immune system cannot produces differ-rent classes of immunoglobulins other than IgM. Therefore, the patients are susceptible to many disorders, infectious diseases and malignancies. Bone marrow or allogeic he-matopoietic cell sorcord blood stem cells transplantation are the two accepted ther-apeutic approaches to treat patients with XHIGM. In addition, various numbers of interventions including IVIG and SCIG therapy, antimicrobial agents and etcare used to control and prevent the XHIGM-related diseases and conditions. In the present manuscript, we review all of these approaches.