Renal cell carcinoma (RCC) accounts for only 3% of adult malignancies, and the lung is the most common site of metastasis of this tumor, which may be accompanied by pleural metastasis. However, solitary pleural involvement is very rare and its presentation with dyspnea as the first manifestation of RCC is extremely rare. We describe a 39-year-old male with episodes of dyspnea dating back 6 months prior to hospital admission. During paraclinical investigations, chest computed tomography (CT) demonstrated pleural effusion and multiple pulmonary nodules, raising the question of primary mesothelioma or metastasis from distant focus. Histopathology and immuno- histochemical examinations of pleural biopsy provided evidence of metastatic RCC of the clear cell type. Therefore, an abdominal contrast computed tomography (CT) was performed, revealing a 3 cm right renal mass, which was then removed by partial nephrectomy. Physicians and pathologists should be aware of unusual presentations of RCC with no symptoms attributable to the kidneys, including dyspnea as in our case.