Alveolar echinococcosis is a rare parasitic disease that is endemic to the northern hemisphere in many parts of the world. Humans are coincidental intermediate hosts infected after eating contaminated food, including fruits and vegetables or being in contact with foxes or dogs. Although most alveolar echinococcal infections are localized to the liver, the larvae may spread to other organs by regional extension or distant metastasis along hematogenous or lymphatic pathways. The liver and lungs are the most commonly affected organs. Alveolar echinococcosis involving the kidneys is very rare. Parasitic infection causes granulomatous response on the host immune system. These lesions may be misdiagnosed as malignant neoplasms. Imaging modalities such as MRI provide useful diagnostic information for the accurate diagnosis of alveolar echinococcosis. In this case, we aimed to present radiological imaging and pathological findings of alveolar echinococcosis mimicking renal cell carcinoma of the right kidney with brain metastasis in a 40-year-old male patient with right lumbar pain.