Background: Inflammatory pseudotumor of the kidney is a very rare benign lesion.
Case Report: In this paper we report a 12 year old girl who had a renal mass raising the suspicion of a malignant neoplasm and pathologic examination revealed an inflammatory pseudotumor
Conclusions: Clinically, IPT mimics malignancy; however, these tumors generally do not recur after complete surgical excision. This is the first reported description in the literature of primary renal inflammatory pseudotumor associated with neurofibromatosis in a child.

Keywords: inflammatory myofibroblastic tumor, inflammatory pseudotumor, Pathology