Vertebral hemangioma is the most common benign tumor of the spine, diagnosed incidentally in most cases. In 0.4% of patients, the lesion is considered aggressive, causing neurological deficits. This subtype of hemangioma is characterized by strong postcontrast enhancement, cortical lysis, and epidural extension causing myelopathy and/or radiculopathy. A 52-year-old man presented with myelopathy symptoms, namely lower-limb hypoesthesia up to the T4-5 sensory level, right leg hyposthenia, and urinary incontinence. Imaging studies revealed a giant dumbbell-shaped lesion causing spinal cord compression, associated with signal alteration of the T3 vertebral body. The diagnosis of schwannoma was not certain given the radiological features, so a biopsy was planned and confirmed the diagnosis of vertebral hemangioma. Preoperative embolization, spinal fusion, and gross-total resection of the extravertebral component of the lesion were performed. This report should raise awareness of the differential diagnosis of dumbbell-shaped spinal tumors and the therapeutic strategies available for aggressive vertebral hemangiomas, a rare lesion that should be managed in a multidisciplinary setting. https://thejns.org/doi/10.3171/CASE24190.