Congenital intracranial immature teratoma in a preterm infant: illustrative case.

backgroundCongenital intracranial immature teratoma is a rare tumor that is present in the first year of life. It is composed of three embryonic germ layers. These tumors are mainly manifested by hydrocephalus. Radiological investigations and histopathological studies are crucial to evaluating and diagnosing intracranial immature teratoma. Few cases were documented in the literature. OBSERVATIONS: In this case, we highlight a preterm infant born with grade III proptosis and an intracranial space-occupying lesion that was diagnosed as high-grade congenital intracranial immature teratoma by brain MRI and histopathology studies. Neoadjuvant chemotherapy was used after the failure of the initial first attempt of craniotomy and lesion resection. Although, the tumor size increased post-chemotherapy, both intracranial and orbital parts of the lesion were excised efficiently with minimal blood loss, achieving complete tumor resection. LESSONS: We demonstrate a case of a rare congenital brain tumor in the pediatric population. We report the diagnostic and treatment challenges besides the effective intraoperative role of neoadjuvant chemotherapeutic agents. To our knowledge, the current case is one of the few published cases of intracranial immature teratoma that presented with ophthalmic manifestations.