The clinicopathological analysis of 250 pediatric cases that had been tissue-diagnosed with renal amyloidosis revealed three patients associated with Henoch-Schönlein syndrome (HSS). The renal biopsies revealed AA-type amyloidosis in all three cases. Case 2 displayed focal and segmental proliferative glomerulonephritis in the same renal biopsy. No evidence of well-known diseases and/or conditions for the development of AA-type amyloidosis except for familial Mediterranean fever (FMF) existed in these particular cases. On the other hand, the frequency of the association between FMF and HSS has been reported extensively in the literature; thus, common etiological factors can be considered. The mechanism involved in amyloid deposition in these cases may be related to HSS-associated chronic antigenemia and/or FMF through a mechanism that is, to date, unknown. Further studies are needed to clarify this causal relationship.