We report an unusual case of lumbosacral osteogenic sarcoma with cauda equina syndrome and invasion into the central venous and cardiac system. A 41-year-old Hispanic man presented to the emergency department complaining of severe low back pain, cauda equina syndrome, bilateral lower extremity edema, and an extra heart sound on physical examination. CT of the lumbosacral spine done in the emergency department demonstrated a sclerotic lesion in the sacrum with cortical destruction, extension into the spinal canal and a bulky soft tissue mass containing calcifications. Supplemental MRI demonstrated marrow replacement of L4, L5, and the sacrum, soft tissue extension of the tumor, and invasion iliac veins extending into the IVC; however, the full extent of the intravascular tumor was not seen on this examination. Surgical laminectomy and biopsy of the spinal tumor provided the diagnosis of osteogenic sarcoma. A transthoracic echocardiogram was performed while the patient was recovering due to nonsustained ventricular tachycardia, which showed an echogenic mass within the right atrium and ventricle. CT pulmonary angiogram confirmed the echocardiogram showing a tumor extending through the pulmonary valve into the main pulmonary artery. The patient underwent en bloc resection of the tumor from the venous and cardiac systems. Histologic examination of the tumor confirmed osteogenic sarcoma. While vertebral osteogenic sarcoma is uncommon, invasion of the spinal canal is common in these tumors. However, tumor extending into the central venous and cardiac system is rare. The previously reported cases of central venous and cardiac involvement have been related to distant metastases or primary cardiac osteosarcomas. There is only one other reported case of direct extension into the venous system by an iliac bone osteosarcoma in an adolescent; however, the tumor did not extend into the pulmonary circulation.