The aberrant or ectopic thyroid in lateral neck is a rare developmental anomaly. Furthermore, the primary thyroid carcinoma arising in ectopic thyroid is extremely rare, only a few cases have been reported so far in English literature. We report a 64-year-old male with left transglottic cancer and primary papillary carcinoma from lateral aberrant thyroid in left lateral neck. Preoperatively, we diagnosed as transglottic cancer with ipsilateral neck metastasis. The patient underwent total laryngectomy, left selective neck dissection and left thyroidectomy. Finally, the patient was diagnosed as left transglottic cancer and papillary carcinoma from lateral aberrant thyroid. Surgeons should take into account a primary ectopic thyroid carcinoma arising in lateral neck may co-exist with another type of head and neck tumor.