C3 glomerulopathy is a recently defined disease category comprising several rare types of glomerulonephritis (GN), including dense deposit disease (DDD), C3 glomerulonephritis (C3GN), and CFHR5 nephropathy. These disorders share a common etiology involving dysregulation of the complement alternative pathway (AP), with genetic defects and/or autoantibodies seen in a proportion of patients. Currently no consistently effective therapy has been found for C3 glomerulopathy; clinical evaluation of agents targeting specific components of the complement system is undergoing. The appropriate timing and duration of proposed therapies need to be further explored. This review focuses on the clinical and histological features, complement tests and treatments of C3 glomerulopathy.