The treatment of common variable immunodeficiency (CVID) is currently based on the early recognition of the condition and replacement immunoglobulin combined with prompt treatment of infections and complications. The route of administration, dose and frequency of administration of immunoglobulin still vary between centres and countries. Other interventions aimed at overcoming the immunological defects in CVID such as interleukin-2 therapy are being studied but there is as yet insufficient evidence to support their routine use. The treatment of complications such as suppurative lung disease uses principles broadly similar to those used for cystic fibrosis, whereas the granulomatous complications involving the lungs and other organ systems are in need of much more research to define optimum therapies.