Drake KA, et al. - Researchers examined histopathology, complement/genetic studies, and renal outcomes of pediatric patients with C3 glomerulopathy (C3G) at their institution, given this rare condition has generally unfavorable yet highly variable consequences. They analyzed all native kidney biopsies done in a single pediatric hospital over a 10-year duration, focusing on characteristics of C3G. Overall 4 patients with dense deposit disease (DDD), 4 with C3 glomerulonephritis (C3GN), and 1 indeterminate case, with characteristics of both C3GN and DDD, were identified. Positivity for one or more nephritic factors (C3NeF, C4NeF, C5NeF) was seen in five patients, with additional positivity for complement factor H autoantibody in 1 patient. A mean follow-up of 33 months revealed progressive renal dysfunction in three patients. Nowadays, complement and genetic investigations are routinely advised for patients with a histopathological diagnosis of C3G. In order to gain more insights into the pathophysiology of this rare disease in children, there is a requirement for careful analysis of these studies and their prognostic and therapeutic implications together with biopsy findings.