Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features. Cisplatin-based chemotherapy was initiated, but rapid growth of the tumor encasing the hepatic artery proper was detected, even after two cycles of chemotherapy. A split LT was carried out, and pathological examination of the explanted liver revealed the involvement of numerous neuroepithelial components, confirming the diagnosis of a Norris grade 3 immature teratoma. The patient recovered well and was discharged on day 19 post-LT. As on date, on postoperative day 240, he has completed seven cycles of a 12-cycle vinblastine and doxorubicin-based adjuvant chemotherapy.