Autosomal dominant polycystic kidney disease (ADPKD) is one of the congenital cystic renal diseases with the highest incidence. ADPKD was suspected of being a risk factor for the emergence of RCC. A 65-year-old male complained of numbness in both knees for a week. The patient came to a neurosurgeon and was advised to perform a lumbosacral MRI. The patient had no complaints. The patient had a history of hypertension but was never treated. Computed tomography intravenous pyelogram (CT-IVP) revealed a heterogeneous lobulated mass in the upper to the middle of the right kidney to the right renal hilus. It also revealed multiple cysts, in both kidneys. The patient underwent an open radical nephrectomy in the right kidney with minimal bleeding. Three years revealed no pain at the surgery site or hematuria. Abdominal MRI revealed no recidive mass. This case report comprehensively described an autosomal dominant polycystic kidney disease that coexists with RCC. malignant lesions were found in ADPKD cases without any clinical symptoms of malignancy. M malignant lesions could be discovered by chance in nephrectomy specimens. Autosomal dominant polycystic kidney disease with renal cell carcinoma is a unique presentation. Despite the rarity of the situation, the patient was successfully treated.