Background Choroid plexus carcinomas (CPC) are rare brain tumors with a dismal prognosis. Although the role of surgery has been well established, the question of whether chemotherapy improves the prognosis is still under discussion. Methods We created a database of all cases of choroid plexus tumors (CPT) reported in the literature up to the year 2004 to determine prognostic factors and different therapeutic modalities. This database was validated by comparison with an existing database of cases until 1997. Results Of 857 documented cases of CPT (median patient age at diagnosis, 3 years), 347 were CPC, 15 atypical choroid plexus papilloma (APP), and 495 choroid plexus papilloma (CPP). Histology was a significant prognostic factor (P < .0001; log rank). Within the subgroup of patients with CPC, both surgery and irradiation were linked to a better prognosis (P < .005). The 104 CPC patients who received chemotherapy had a statistically better survival than those without chemotherapy (P = .0004). When subgroups were defined by radiation treatment, chemotherapy remained beneficial in the subgroup of nonirradiated tumors (P = .0001). The benefit of chemotherapy was also significant when the analysis was restricted to the subgroup of patients with less than completely resected CPC (2-year overall survival (OS) 54.8 ± 7% (standard deviation (SD) vs. 24.4 ± 7%, P < .0001) and when this subgroup was further divided into smaller subgroups. Likewise, in a multivariate analysis, chemotherapy was highly significantly linked to better prognosis (P = .0001). Conclusion Patients with less than completely resected CPC should receive chemotherapy.