We report the case of an 18-yr-old man with quiescent ulcerative colitis complicated by concomitant primary sclerosing cholangitis and rapidly progressive glomerulonephritis. Findings on immunofluorescence microscopy and electron microscopy suggested that glomerular injury occurred secondary to the deposition of circulating immune complexes. Renal disease responded to treatment with corticosteroids. A review of the literature found similar cases of glomerulonephritis and inflammatory bowel disease, but no previous association with sclerosing cholangitis has been recognized. The pertinent clinical, immunological, and pathophysiological aspects of this association are reviewed.