Objective: The Pemberton sign is related to venous obstruction in patients with goiters. It has been mainly reported in patients with euthyroid and hyperthyroid goiters and is rarely seen in hypothyroidism. Methods: We present the case of a 52-year-old male with hypothyroidism due to Hashimoto thyroiditis who developed cervical compression with an impressive Pemberton sign. Results: A 52-year-old male was diagnosed with primary hypothyroidism due to Hashimoto disease based on positive peroxidase antibodies, high thyroid-stimulating hormone (TSH) and low free thyroxine (T4). His initial complaints were fatigue and neck discomfort. He was initially treated medically with levothyroxine without improvement and referred to endocrinology for further management. Physical examination at the initial endocrine visit was pertinent for an impressive Pemberton sign. Because the patient was interested in medical therapy, levothyroxine was increased and liothyronine was added. He eventually underwent total thyroidectomy due to worsening neck discomfort and breathing difficulties, which alleviated all his symptoms. Conclusions: The Pemberton sign is elicited by a simple maneuver and is very useful for identifying patients who may require thyroidectomy for compressive symptoms.