A 30-year-old woman with chronic renal failure (CRF) due to glycogen storage disease Type I (GSD I) was admitted for dialysis. Hemodialysis (HD) was introduced as the primary therapeutic modality. However, maintenance HD was very difficult to conduct because of hypotension during the HD sessions. Furthermore, hypoglycemia and metabolic disturbances persisted. After changing from HD to CAPD, fasting blood sugar was significantly elevated through a continuous glucose supply from the dialysate. The values of ketone, non-esterified fatty acid, blood urea nitrogen/creatinine (BUN/ Cr), and glucagon were improved. CAPD not only controlled uremia, but also ameliorated the metabolic disturbances of GSD I. Therefore, we conclude that CAPD is superior to HD as a dialytic modality for patients with CRF due to GSD I.