Homozygous deficiency of the second component of complement (C2) occurs in one in 10, 000 individuals. Its clinical manifestations range from essentially no symptoms to recurrent infections or evidence of collagen‐vascular disease. We present here a case report and a review of the literature focusing on recurrent systemic infections in C2‐deficient individuals. Thirteen of 20 patients vi'ith C2 deficiency and a history of invasive bacterial infections have had recurrent episodes of bacteremia or meningitis. Most of these patients were children, andStreptococcus pneumoniaewas the most common pathogen reported. The use of prophylactic antibiotics.Haemophilus influenzaetype b and pneumococcal vaccines, and prompt medical attention for any febrile illness should be encouraged in children with documented C2 deficiency. Although there are no studies to date to document their efficacy, the above measures may serve to diminish the frequency of recurrent systemic bacterial disease in these child