INTRODUCTION: Sacrococcygeal teratoma is commonly a benign tumor that arises in the sacrum and base of the coccyx. It has an incidence of 1 in 40 000 and a female-to-male ratio of 4:1. Computed tomography scan and magnetic resonance imaging (MRI) are key to diagnosis and surgical resection is the definite treatment. CASE PRESENTATION: A 10-year-old female presented with a gradually progressive sacral swelling since birth associated with fever, pain, and discharge from the swelling for 2 weeks. On examination, there was an approx. 10 × 11 cm mass in the sacral region with a pus point and signs of inflammation were present. On palpation the mass was tender. MRI lumbosacral spine showed a complex mass in the infra-coccygeal region with features suggestive of sacrococcygeal teratoma. Surgical resection of the tumor was performed. DISCUSSION: Sacrococcygeal teratoma arises from totipotent cells from Hensen's node at the coccygeal region. Clinical presentation varies according to type with common manifestations being progressive swelling at the coccygeal region and features of mass effect such as low back pain, and bowel or bladder symptoms. Imaging helps in diagnosis as well as detecting the size, and extent of the tumor as well as its relation to nearby structures. Surgery via transabdominal or trans-sacral approach is the curative treatment. CONCLUSION: Diagnosis of sacrococcygeal teratoma should be considered if a patient presents with gradually progressive swelling of the sacral region.