Background: Choroid plexus carcinoma is a highly aggressive malignant, infrequent tumor with poor prognosis. About 80% of choroid plexus carcinoma occurs in children, but it is uncommon in adults. Because of the rarity of the choroid plexus carcinoma, there is no established treatment protocol for this malignancy. Case Description: A 21-year-old man with past medical history of asthma presented to us with the chief complaint of morning headache for one month. His brain magnetic resonance imaging (MRI) showed a mass in the trigone and occipital horn of the left lateral ventricle. He had undergone a left occipitoparietal craniotomy, posterior interhemispheric precuneus approach with grossly total removal of the tumor. The histology examination of the tumor proved to be choroid plexus carcinoma. This patient achieved a favorable outcome after having a grossly complete surgical resection followed by postoperative adjuvant radiotherapy and chemotherapy. Conclusions: Choroid plexus carcinoma is aggressive and is associated with dismal prognosis. The 5-year survival rates for choroid plexus carcinoma vary between 10% and 50%. Currently, there is no established treatment protocol for choroid plexus carcinoma. Complete resection of this malignant tumor is the primary goal of treatment since it allows best chance of survival and improves the overall prognosis.