Fourty-four children with Henoch-Schoenlein nephritis were studied at the onset of the nephropathy and during a follow-up from 6 to 110 months. The extra-renal manifestations were purpura (100%), abdominal pain (63.5)% or melena (27%), arthlagias (61.5%), neurological symptoms with convulsions (4.5%) and retinal involvement (4.5%). The clinical presentation of the nephropathy consisted in haematuria and proteinuria (41%), isolated haematuria (30%), acute renal failure (ARF) (23%), nephrotic syndrome (4%) or isolated proteinuria (2%). Hypertension was present in 17 patients. Renal biopsy was performed in 18 patients and the glomerular changes were graded according to the classification of ISKDC; the renal histopathology ranged from minimal lesions to severe crescentic glomerulonephritis and was found to correlate with clinical state. Twenty-four patients, who showed severe clinical presentation and/or diffuse mesangial proliferation with high proportion of crescents, received a corticosteroid therapy. Most of our patients followed a relatively benign course: all but one of patients with ARF have normal renal function at the end of follow-up and no patients with less severe renal presentation got a bad outcome. Only 2 patients showed relapse of nephropathy and purpura at the 6th and 8th year of follow-up, respectively. After 24 months of follow-up the clinical outcome of a group of 19 patients receiving corticosteroid therapy was not very different from that of 11 untreated patients.