Academy of Medical Sciences of I.R. Iran Archives of Iranian Medicine 1029-2977 29 2 2026 02 01 Primary Chondroblastic Osteosarcoma of the Kidney: A Case Report and Review of Literature 123 128 10.34172/aim.34620 EN Recep Bedir https://orcid.org/0000-0001-8247-3781 Selim Yazar https://orcid.org/0000-0002-9843-9008 Journal Article 10.34172/aim.34620 2025 06 03 2025 10 06 The primary renal chondroblastic variant of osteosarcoma is an exceedingly rare and aggressive malignancy with poor prognosis. This report details the case of a 40-year-old female presenting with flank pain diagnosed with primary renal osteosarcoma. We outline the clinical, radiological, and histopathological features of this tumor, along with current therapeutic strategies.