OBJECTIVES: This literature review aimed to gather and narratively synthesize evidence on the clinical, economic and humanistic burden associated with C3G, which encompasses dense deposit disease (DDD) and C3 glomerulonephritis (C3GN). METHODS: EMBASE and Medline databases were searched for relevant English-language publications, which were selected based on pre-defined inclusion criteria through a two-step screening process; (i) abstract and (ii) full-text screening. RESULTS: The review identified 33 heterogeneous publications, that varied with respect to the number of patients included (5 –168); study settings (single-center [n=22] vs. multi-center [n=10] vs. database [n=1]) and geographies (North America [n=12], Asia and Europe [n=9 each] and other regions [n=3]). Sixteen publications were targeted to DDD patients, 4 to C3GN and the remaining 13 included both types. All publications provided evidence on clinical burden, none had evidence on economic or humanistic burden. At diagnosis, there were no major differences between C3GN and DDD patients. Mean age at presentation was in the range 6.8–36 years and mean proteinuria levels ranged from 1.25– 5.1 g/day. Up to 30% of C3GN (follow-up ~10 years) and 100% of DDD patients (follow-up ~15 years) progressed to end-stage renal disease (ESRD). In both conditions, histological presentations of glomerular crescents and interstitial fibrosis were associated with progression to ESRD, along with complement gene mutation, low glomerular filtration rate at biopsy and no remission in proteinuria (P<0.01 each). Among C3GN and DDD patients with ESRD, renal transplants were as high as 59% and 92% respectively. Disease recurrence was highly prevalent in transplanted patients. CONCLUSIONS: The high clinical burden associated with C3G could be attributable to lack of interventions to slow or arrest disease progression. Furthermore, the absence of evidence on the humanistic and economic burden associated with C3G is indicative of a need for further research.