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Inflammatory pseudotumors are uncommon benign tumors of unknown etiology which may develop at several anatomical sites, e.g., the airways and gastrointestinal tissues, soft tissues, the orbit, the spleen, or the lymph nodes. The renal site is extremely rare, and presents the problem of differential diagnosis as the clinical and radiological aspects of this tumor are similar to those of an adenoma or an angiomyolipoma, and suggest the presence of a carcinoma, in particular a cystic renal carcinoma which is also a rare form of tumor. There is therefore a risk that this benign lesion could be misdiagnosed. Due to the good prognosis associated with this type of tumor, in cases where the definitive diagnosis has been established no surgical procedure is necessary. However, the difficulty in making this diagnosis preoperatively means that in general the organ has to be surgically removed so that a histological analysis can be made and the negative or positive findings confirmed. In the present study, the case of an inflammatory pseudotumor of the kidney has been described. In this instance, radical nephrectomy of the left kidney was carried out as the disease was presumed to be renal cell adenocarcinoma. However, the histopathological analysis was negative as regards malignancy, and indicated the presence of an inflammatory pseudotumor. This article raises the question of the problem in establishing a preoperative definitive diagnosis, as a correct diagnosis is often only confirmed following nephrectomy (in cases where the contralateral kidney is healthy).