Lymphangiomas are benign lesions consisting of abnormal proliferations of lymphatic vessels. Lymphangiomas associated with bone involvement, particularly in vertebral bodies, accompanied by cord compression, are extremely rare, and our literature review yielded only a few relevant reports. We describe a 61-year-old man presenting with progressive paraparesis and sphincter disturbance of 5 months’ duration. Magnetic resonance imaging (MRI) revealed an enhancing T8 vertebral body involvement as well as a homogeneously enhancing posterior epidural mass at the T7–T8 level, with severe cord compression and cerebrospinal fluid (CSF) blockade. The patient underwent surgery via T7–T8 laminectomy, and after the removal of the epidural mass, the surgical procedure was stopped due to severe bleeding. Histopathologic examination reported a lymphangioma. After 10 days, the patient was able to walk. In the sixth postoperative month, MRI showed complete relief of the mass effect. The wide spectrum of the preoperative differential diagnosis of lymphangiomas renders a definite preoperative diagnosis impossible; therefore, histopathologic examination is the sole definite route for their diagnosis. In case of the solitary lymphangiomas of the spine with epidural compression, preoperative angiography and embolization should be considered to reduce intraoperative bleeding and enhance the chance of total resection and total surgical resection should be performed to decrease the likelihood of recurrence.