Primary intrarenal/perirenal neuroblastoma (NB) is NB that primarily arises in intrarenal and/or perirenal regions. Regarding its location, this tumor can mimic Wilms' tumor a more common pediatric renal tumor at presentation. Owing to diference in clinical management andprognosis, it is crucial to distinguish primary intrarenal/perirenal NB from Wilms' tumor at the time of diagnosis. Recognition of its characteristic features, which are distinctive from its adrenal counterpart, is helpful to guide to the correct diagnosis and proper treatment. However,; due to its rarity with less than 100 cases described in English literatures, the characteristics of primary intrarenal/perirenal NB have not been widely studied The authors, therefore, report this case of primary intrarenal/perirenal NB, which occurred in right kidney of a 5-year-old Thai girl in order to illustrate the characteristic features of this tumor To the authors'knowledge, this case is the first case ofprimary intrarenal/perirenal NB that has been reported in Thailand