Retrosternal goiters constitute roughly 1–3% of all goiters and are slow growing in nature. Although several classifications and definitions are described, they usually signify greater than 50% of thyroid mass being present substernally. Presentation can vary from neck swelling to frank compressive symptoms, with dyspnea on exertion being the most common. Due to these vague symptoms, predominantly retrosternal goiters with a small cervical component are often misdiagnosed as chronic obstructive airway diseases. Clinical examination supplemented by cross-sectional imaging of the neck and chest is crucial to establish diagnosis and quantify the degree of retrosternal extension. This also yields key information regarding a suitable surgical approach, the need for sternotomy, and the potential risk to recurrent laryngeal nerve and important vascular and visceral structures. Surgical excision is the mainstay of treatment and should be planned with a multidisciplinary team, including a thyroid surgeon, cardiothoracic surgeon, and anesthetist. Surgical adjuncts such as intraoperative nerve monitoring and parathyroid fluorescence imaging can prove useful and should be employed whenever possible.