IgA nephropathy and IgA vasculitis with nephritis (IgAVN), albeit rare, represent two relatively frequent glomerular conditions in childhood. Compared to adults, pediatric IgA nephropathy has a more acute presentation, most frequently with synpharyngitic macrohematuria, and histologically with more intense inflammation and less intense chronic damage. Management of these conditions is controversial, and supported by little high-quality evidence. Paucity of evidence is due to the disease heterogeneity, to its inter-ethnic variability, and to the difficulty of extrapolating data from adult studies due to the peculiarities of the condition in children. IgA vasculitis with nephritis is a renal manifestation of a systemic disorder, typical of the pediatric age, in which both the diagnosis of kidney involvement and its management are poorly defined, and an interdisciplinary approach is crucial. Both conditions can have a profound and long-lasting impact on kidney function and the global health of affected children. This presentation will discuss the diagnosis and management of these conditions, focusing on the differences arising from current evidence.