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We reviewed retrospectively 31 cases of candidemia in children with central venous catheters. Infection rate was significantly higher in 1− to 4-year-old children than in other age groups (8.4% vs. 2.2%; P < 0.05). Serious sequelae occurred in 11 (35%) cases and included fatal outcome (5 instances), Candida endocarditis (2), renal abscesses, meningitis, arthritis and osteomyelitis (1 each). Complications were significantly more common in infants than in older children (P < 0.05) and appeared 3 to 52 days after the first positive blood culture (mean, 16 days). In fatal cases catheters were left in place a significantly greater number of days than in nonfatal cases (P < 0.05). A literature review identified 43 additional cases of catheter-related candidemia described in 11 series. The rate of Candida infection in the group as a whole was 2.7%. Patients treated with catheter removal plus amphotericin B had a significantly higher cure rate then patients treated with catheter retention plus amphoteriein B (P = 0.009). Prompt catheter removal remains crucial in the treatment of catheter-related candidemia.