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 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 less than 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 less than 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 less than 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 amphotericin B (P = 0.009). Prompt catheter removal remains crucial in the treatment of catheter-related candidemia.