C3 glomerulopathy is a kind of glomerular diseases mediated by abnormal activation of alternative complement pathway. As diversity and multiplicity of pathogenic mechanism, heterogeneity exists in the clinical manifestation and pathological features of C3 glomerulopathy. The clinical manifestation of the disease may be shown as abnormality in urine, hypertension, hematuria, nephrotic syndrome, nephritic syndrome, renal insufficiency, etc. Membranoproliferative glomerulonephritis, mesangial proliferation, crescent formation, focal segmental necrosis, diffuse hyperplasia and exudative lesions, etc may be found in renal biopsies. Also, the prognosis of C3 glomerulopathy is not uniform. The clinical manifestations and pathological features of C3 glomerulopathy were reviewed in the present paper. DOI: 10.11855/j.issn.0577-7402.2014.12.15