Sacrococcygeal teratoma (SCT) is a rare tumour with a low incidence, but it is the most common extragonadal tumour in the newborn. It occurs at the base of the coccyx (tailbone) and is derived from embryonic germ cell layers. Although the majority are benign in nature, they are associated with significant morbidity and mortality for the mother and the foetus. A multidisciplinary approach is needed where an accurate early diagnosis should be made antenatally, leading to anticipation of complications during intrapartum for the mother and foetus, and ultimately early, complete surgical excision of the tumour. The prognosis for survival is good after resection. We report a case of a newborn delivered at 29 weeks who was diagnosed with SCT at post-partum but unfortunately passed away due to the complications of prematurity before surgery could be performed. This case report highlights the literature review of this pathology, especially the management of SCT, which begins in the antenatal period soon after the diagnosis is made.