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We describe a case of primary pneumococcal peritonitis which uncovered a family with compound heterozygous deficiency for complement factor I. Primary pneumococcal peritonitis has been described in women but has not been previously linked with complement disorders. We propose that unexplained pneumococcal peritonitis should be considered a “red flag” for primary immunodeficiency.

Primary pneumococcal peritonitis can be the first presentation of a familial complement factor I deficiency 1