Primary sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) of the thyroid gland is a very rare disease. We present the clinical and histopathologic findings of a 37-year-old woman recently diagnosed with SMECE of the thyroid gland. The patient, clinically euthyroid, who presented with a neck swelling since last 2 years along. Fine needle aspiration cytology suggested thyroid papillary carcinoma. Total thyroidectomy, central neck dissection and right selective neck dissection were performed. Although SMECE is considered to be a relatively slow growing and non-aggressive tumor, occasional metastasis does occur. We report an additional case of SMECE, with metastasis to regional lymph nodes. Physicians should be aware of extended operation, including total thyroidectomy and/or neck node dissection for metastatic lesion of the neck node. More standardized treatment is likely to evolve in the future.