Introduction: Choroid plexus carcinoma is an uncommon/rare cause of a hemispheric cerebral tumor arising from lateral ventricles. Discussion: The exceedingly low prevalence of CPC in children has hampered the creation of standardized clinical trials, leaving therapy options to expert opinion and case studies. Surgery, chemotherapy, radiation, and autologous hematopoietic cell rescue are all options for treatment. Conclusion: In rare conditions like these possible evidence-based treatment has low evidence and further establishment of more data-based studies are to be done to further out more options and adjuvant therapies for the future.