Hemophagocytic syndrome, splenic microabscesses and pulmonary cavitary lesions were presented in a 17-month-old boy with prolonged fever, hepatosplenomegaly and a history of tuberculous lymphadenitis. Clinical course mimicked tuberculosis. Blood cultures were negative. Ultrasound-guided, percutaneous aspiration from splenic microabscesses grew Burkholderia cepacia. He was treated successfully with trimethoprim-sulfamethoxazole. This child with chronic granulomatous disease had an unusual clinical manifestation of B. cepacia infection.
