Metanephric adenoma is a rare benign kidney tumor with a favorable prognosis. These neoplasms are often interpreted as malignant at the preoperative stage, since they do not have specific characteristics showing during instrumental imaging methods, which leads to excessive surgical treatment in the context of nephrectomy volume. Therefore, histological verification is the gold standard for diagnosis. The morphological features of metanephric adenoma are in many ways similar to such kidney tumors as papillary renal cell carcinoma and nephroblastoma (Wilms’ tumor), so sometimes additional research methods are required, including immunohistochemical and molecular genetic ones. In this article, we report a rare clinical case of metanephric adenoma in a forty-seven-year-old woman. The results of clinical and instrumental exams, as well as morphological features of the tumor are presented. A review of the literature on the clinical, morphological, and molecular aspects of this tumor is also presented.