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We report a rare case of a mediastinal goiter confined to the thoracic inlet and cavity presenting with ventricular tachycardia as the sole clinical manifestation.The patient did not have any of the typical features of a mediastinal goiter such as neck swelling, dysphagia, or respiratory difficulty, but instead had spontaneous onset of wide-complex tachycardia requiring emergency treatment. This atypical presentation led to initial misinterpretation of imaging studies and delayed diagnosis of the mediastinal mass. The large, completely intrathoracic thyroid goiter abutted the cardiac muscle and required a combined transcervical and median sternotomy approach for removal. The arrhythmia resolved postoperatively.To our knowledge, this case represents the first documented presentation of ventricular tachycardia as a unique and sole feature of mediastinal goiter.