A previously healthy 16-month-old boy presented with 5 days of fever, a limp favoring his right side, and swelling and erythema of the dorsal left foot and left great toe. Contrasted magnetic resonance imaging revealed septic arthritis of the left knee, the interphalangeal joint of the left great toe, and the left subtalar and ankle joints. He had osteomyelitis of the left talus and intraosseous abscess formation in the left calcaneus. Cultures of the blood, left knee synovial fluid, and abscess fluid all showed growth of Pseudomonas aeruginosa. He was subsequently diagnosed with X-linked agammaglobulinemia with a novel mutation in the Bruton’s tyrosine kinase gene. He had a promising recovery after intravenous immune globulin and antimicrobial therapy. Unusually severe infections in children, particularly with atypical organisms, should prompt suspicion of an underlying immunodeficiency.

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