BACKGROUND: Chronic meningococcemia, defined as a meningococcal septicemia without meningeal symptoms with persistence of fever for at least one week prior to any antibiotics, is uncommon. Its pathophysiology remains unclear and a defect in host immunity has been suggested. CASE REPORT: A 15 year-old adolescent was examined because he suffered from fever for 6 days, headache, arthralgias. A disseminated erythema led to consider the diagnosis of vascularitis that was confirmed by skin biopsy. At day 9, blood culture yielded Neisseria meningitis group B that was confirmed by a second blood culture; the CSF was normal and sterile. The patient was given ceftriaxone plus penicillin for 14 days and completely cured. A detailed analysis of the complement system was negative but the patient was found to be deficient in IgA. CONCLUSION: This is the first reported case in which chronic meningococcemia is associated with complete IgA deficiency.