Comprising a very small percentage of all teratomas and all intracranial tumors, intracranial teratomas are extremely rare; however, they are the most common tumors among fetal intracranial tumors. The outcome of these fast-growing aggressive tumors has been reported to be generally poor and the long-term survival rates are seen only in a small number of patients. We present 5-year and 10-month-old girl who wasprenatally diagnosed with a congenital intracranial teratoma arising from the left lateral ventricle and treated successfully by several surgical removals and adjuvant chemotherapy during the first year of life. Final histopathologic examination showed a teratoma consisting of mature and immature tissue elements at the resected specimen. A rare histopathologic feature for intracranial teratomas "nephrogenic differentiation" was also noted. Herein, a prolonged clinical course was presented along with the radiologic features and the outcome was discussed by a review of relevant literature in a congenital intracranial teratoma case.