Sacrococcygeal chordoma is a rare, locally aggressive malignant tumor originating from remnants of the notochord, and it represents the most common primary malignancy of the sacrum. This review provides an in-depth discussion of the clinical presentation, pathological features, imaging characteristics, and management of sacrococcygeal chordomas. These tumors typically present with local pain, neurological deficits, and a palpable mass, and they are known for their high recurrence rate and challenging surgical management. Imaging, especially MRI, is crucial for accurate diagnosis and surgical planning, given its ability to delineate the extent of the tumor and its relationship with surrounding structures. Complete surgical excision remains the cornerstone of treatment, with prognosis largely depending on the ability to achieve negative surgical margins. We present a case of a 45-year-old male who presented with bowel and bladder dysfunction and a painful, progressively enlarging mass over the sacrum. MRI revealed a large, well-defined soft tissue mass involving the distal sacrum and coccyx, extending into the retrorectal space. The patient underwent successful en bloc surgical resection, and histopathological examination confirmed the diagnosis of chondroid chordoma.