Renal-Cell Carcinoma (RCC) accounts for 2% of all cancers. Metastases are present in up to 30% of patients at diagnosis or appeared during follow up [1]. Atypical RCC metastases are sites, other than chest, liver, bone, adrenal, brain, and nodes, and their presentation is very rare. We describe the case of a 71-years-old male, who was submitted to a left laparoscopic radical nephrectomy for Clear Renal Cell Carcinoma (CRCC). After a 14-year follow-up, MRI scan showed a 5-cm solid lesion of the perineum, that originates from the right corpus cavernosum. A percutaneous trans-perineal biopsy revealed an atypical metastasis of CRCC. To reduce surgical risk during the exeresis of the mass, the patient received 4 cycles of pazopanib as presurgical treatment, with a shrinking of the lesion at follow-up MRI scan. He underwent a complete resection of the residual mass. Histopathology confirmed the diagnosis of atypical corpus cavernosum metastasis of CRCC. Presurgical therapy with Target Molecular Therapy (TMT) is feasible and might have several potential advantages for patients in case of complex metastasectomy.