Twelve patients with nephrotic syndrome (NS) in Henoch-Schoenlein (HS) nephritis were treated with a high dose of intravenous methylprednisolone (MP) on each of nine alternate days followed by oral prednisolone for 4 to 6 months. Renal biopsy was performed on 10 of the 12 patients. The glomerular change in 5 patients, which was accompanied by crescents and/or sclerosis, with NS and acute nephritic syndrome (ANS) at onset, was more severe than that of the other 5 patients with NS and hematuria at onset. The renal insufficiency or hypertension in these 5 patients with NS and ANS improved within 2 weeks on this MP therapy. After a mean follow-up period of 40.5 months, all patients except 2 revealed normal physical findings and renal function as well as urinary findings. Repeated biopsies in the 2 patients with NS and ANS at onset demonstrated an improved renal pathology in comparison with their initial biopsies. No severe side effects related to high-dose intravenous MP followed by oral prednisolone therapy were encountered in any of the patients. Our results suggest that high-dose intravenous MP therapy can lead to a favorable outcome in patients with NS in HS nephritis.