We have inserted 20 totally implantable central venous devices in 17 patients with severe metabolic disease over a 43-month span. Patient ages ranged from 2 months to 17 years (mean, 4.2 years). The underlying pathology was Gaucher's disease in six patients, vitamin D-dependent rickets type II in five, propionic acidemia in two, and methylmalonic acidemia, 3-hydroxyl-3-methylglutaryl coenzyme A (CoA) lyase deficiency, fructose 1,6 diphosphatase deficiency, and urea cycle disorder in one child each. There were seven complications (six due to catheter-related infection and one due to occlusion of the system) during a total of 7,278 patient-catheter days. The infection rate was 0.8 per 1,000 days. Six catheters were removed due to complications and two due to completion of treatment. There were no operative complications or deaths. Our experience demonstrates that a totally implantable device may be useful in children with metabolic disease who need long-term venous access. Attention should be given to minimize the infection rate to reduce the rate of catheter removal.