The upper urinary tract is the most common human organ system affected by congenital anomalies. A Horseshoe kidney is a fusion anomaly, it can be described as a fusion across the midline of 2 distinct functioning kidneys. The incidence of renal tumors in a Horseshoe kidney is higher than in the normal population. We present a 60-year-old male patient with a history of Horseshoe kidney and a diagnosis of clear cell renal cell carcinoma who underwent a combined therapeutic approach, guided by interventional radiology. This approach involved selective transarterial embolization and microwave ablation. Three months after surgery and with abdominal MRI follow-up, there is evidence of a non-viable tumor, indicating a favorable response to the intervention.