Pediatric vasculitis is a complex group of disorders that commonly presents with multisystem involvement. Renal vasculitis can be isolated to the kidneys or can occur as part of a broader multiorgan vasculitis. Depending on severity, renal vasculitis may present as acute glomerulonephritis (AGN) often associated with hypertension and sometimes with a rapidly deteriorating clinical course. Prompt diagnosis and initiation of therapy are key to preserving kidney function and preventing long-term morbidity and mortality. This review focuses on the clinical presentation, diagnosis, and treatment objectives for common forms of renal vasculitis seen in pediatric patients.