Sacrococcygeal teratoma is one of the most common pediatric tumors. In contrast, its highly developed variant known as Fetiform Teratoma is an extremely rare elusive subtype with overall incidence of 0.01% and reported in only 35 cases worldwide.

This is a case report of a large sacrococcygeal fetiform teratoma in a 3-year-old female from the Philippines seen at the Outpatient Department presenting with history of sacrococcygeal mass initially noted since birth. Work-ups revealed normal tumor markers and a large multi-lobulated, heterogenous and hypervascular Altmann type I sacrococcygeal teratoma on imaging. Preoperative endovascular angioembolization with subsequent En Bloc Excision and coccygectomy was performed. The tumor was confirmed to be fully excised, and no malignant or immature features were found on histopathological examination. Early diagnosis, meticulous preoperative preparation, complete surgical excision, involvement of multidisciplinary team, active surveillance and long-term follow-up are key in the management of sacrococcygeal tumors. Utilization of preoperative endovascular angioembolization is an innovative, safe, feasible, and beneficial in minimizing perioperative blood loss and improving outcomes for large pediatric sacrococcygeal tumors.