This case report highlights both an extremely rare form of goiter presentation with venous compressive symptoms and also some challenges in the treatment of such extreme cases. Compressive symptoms secondary to thyroid gland enlargement are related in most cases to its position in the thoracic inlet. Retrosternal goiter incidence varies widely depending on its definition but can be found in up to 45% of all thyroidectomies. A 57-year-old man presented with goiter and features of superior vena cava syndrome. Neck imaging by computed tomography (CT) showed an increased thyroid gland volume centered to its right lobe and isthmus measuring 87 × 57 × 110 mm with bilateral compression of the venous brachiocephalic trunks as well as left side cervical tracheal deviation. Thyroidectomy was complicated by hemorrhagic shock secondary to laceration of the anterior jugular and thyroid veins during thyroid gland mobilization. The patient was monitored in an intensive care setting during the early postoperative period and was eventually discharged on postoperative day 5. Symptomatic bilateral brachiocephalic venous compression is an extremely rare form of presentation of goiter. Thyroid gland enlargement with compressive symptoms should prompt a thorough evaluation of neighboring structures, and preoperative planning is key to minimizing complications. Life-threatening complications can occur in complex cases such as this one. A rare presentation of goiter with some features of superior vena cava syndrome secondary to external bilateral compression of the venous brachiocephalic trunks is presented. Some technical challenges concerning the surgical approach are also discussed, with a focus on intraoperative bleeding due to venous congestion of cervical and thyroid veins.