Carcinosarcoma of the parotid gland is a rare and aggressive malignant mixed tumor in which carcinomatous and sarcomatous elements coexist and they may metastasize together or independently. The tumor may occur in patients with previous or coexisting pleomorphic adenoma or may arise de novo of the parotid gland. We report a case of 56-year old male with history of carcinosarcoma of the parotid gland that subsequently developed solitary brain and lung metastases. The patient underwent craniotomy of the metastatic and symptomatic brain lesion and had adjuvant radiosurgery postoperatively. After that, his headache disappeared and the left hemiparesis improved. The next-generation sequencing gene study identified the amplification of IRS2, CDK4, IGF1R, MDM2 and MYC genes in metastatic brain lesion. In primary parotid lesion, genomic alteration identified amplification of RAF1, CDK4, IGF1R, MDM2 and MYC genes. The patient then entered a clinical trial and did not show any improvement. Due to the brain mets re-growth, a 2nd craniotomy was performed. He again improved significantly postoperatively. He currently received salvage chemotherapy for his progressive lung metastasis. The prognosis of carcinoma with distant metastasis is poor and the standard treatment has not been established yet. Herein we will present detailed pathological and molecular abnormality profiles of this case and will review similar cases reported in literatures. To our knowledge, this is the first case with carcinosarcoma of the parotid gland that developed brain and lung metastasis.