Renal cell carcinoma (RCC) represents the most common solid malignancy of the kidney, comprising a broad spectrum of histopathological entities. Advances in diagnostic imaging, histopathological classification, and minimally invasive surgical techniques have improved early detection and treatment options. However, renal cell carcinoma with sarcomatoid dedifferentiation remains a challenge due to its aggressive nature and resistance to systemic therapies. We report the case management of a 69-year-old male with a history of significant comorbidities diagnosed with an advanced right renal cell carcinoma cT3aN1M0 who underwent a laparoscopic radical nephrectomy (LRN) and lymph node dissection with minimal blood loss in 110 minutes of surgery. The patient’s postoperative recovery went well, with no significant complications. Histopathological results revealed a renal carcinoma with sarcomatoid and rhabdoid dedifferentiation staged as pT3aN1, with metastases identified in two out of four retrocaval lymph nodes. This case underscores the feasibility of minimally invasive surgery in advanced renal cancer and the prognostic implications of aggressive histological subtypes.