Ectopic lingual thyroid is a rare developmental anomaly. It is caused by aberrant embryogenesis during the thyroid descent to the neck. It may remain asymptomatic or present with dysphagia, hemoptysis, dyspnoea or dysphonia. Clinically, it presents as a mass lesion on the base of the tongue. The most important diagnostic tool for an ectopic lingual thyroid is the 99mTc radionuclide scan, but imaging modalities such as computed tomography scan and magnetic resonance imaging may also help to assess its location and extent and to rule out the presence of normal thyroid tissue in the thyroid bed. The management of an ectopic thyroid remains controversial. No treatment is required for asymptomatic patients in the euthyroid state. Patients with hypothyroidism should be treated with thyroid hormone substitution therapy. Malignant transformation is an indication for complete surgical resection. Ablative radioiodine therapy can be considered for older patients and those who are unfit for surgery. In complicated cases, surgical resection is recommended through the transoral, transhyoid or lateral pharyngectomy approach. We report a case of ectopic lingual thyroid in a 35-year-old man who presented with nasal twang and hemoptysis.