Primary adenoid cystic carcinoma (ACC) of the upper anterior mediastinum mimicking a thyroid tumor has rarely been seen in clinical practice and lacks a standard of care therapy. Here, we report a 47-year old female patient with an ACC originated from the upper anterior mediastinum presenting as a thyroid gland tumor. The patient received gross surgical resection of the tumor and underwent post-surgical chemotherapy and radiotherapy. The patient was free from local recurrence 3-years following initial treatment, but developed multiple lung metastases. She remains under clinical observation without discomfort and is still followed as an outpatient. Here, we also summarized recent reports of similar cases with hope to provide some experience for future clinical practice.