The authors present a rare case of papillary carcinoma arising from the pyramidal lobe of the thyroid in a 54-year-old woman, who presented with a right submental palpable mass. An ultrasound evaluation depicted a 3 cm mixed echoic mass from the thyroid cartilage level without a focal lesion in the thyroid gland. Surgical specimens obtained during bilateral thyroidectomy confirmed papillary carcinoma of the pyramidal lobe. To the authors' knowledge, this is the first case report to describe papillary carcinoma arising from the pyramidal lobe of the thyroid gland.