Lipomas are the most common soft tissue tumors, accounting for half of all soft tissue masses. However, intravascular lipomas (IVLs) are rare. IVLs have been described in case reports in the various major veins. They are often asymptomatic and found incidentally. Given the risk of malignancy, as liposarcomas are the second most common soft tissue sarcoma in adults and account for 1 % of all malignancies, histological differentiation is important, and surgical excision remains the mainstay of treatment in the literature. We present the case of a 61-year-old male who presented with left chest pain that radiated to his left shoulder and arm. On further evaluation, the patient endorsed left-hand swelling and weakness and increased prominence of veins in the left upper extremity. He underwent a CT scan that showed a hypodense mass with fat attenuation in the left innominate vein extending extra-vascularly and into the left internal jugular vein, measuring 2.3 × 2.0 × 5.2 cm. Successful surgical excision and reconstruction of the innominate vein with reimplantation of the left internal jugular vein was performed through median sternotomy and left cervical incision. The postoperative course was unremarkable, and the patient recovered well. The clinical presentation of an intravascular lipoma in a large caliber vessel can be subtle and nonspecific. This case report characterizes this rare vascular tumor and includes a contemporary literature review, summarizing the diagnosis and treatment options.