Background: Liposarcomas of the thyroid gland are extremely rare tumors, and, to our knowledge, only 12 cases have been reported in the English literature. An accurate diagnosis is challenging due to the nonspecific clinical presentation of this cancer, frequently defined just by a swelling of the neck. Patient Findings: We present an 82-year-old woman with liposarcoma of the thyroid, complaining of a fast-growing neck mass. MRI and neck ultrasound showed a large lipomatous mass, which corresponded to a cold nodule in the thyroid scan. After performing a total thyroidectomy, the diagnosis of a well-differentiated liposarcoma of the thyroid gland was made, showing an MDM2 amplification in fluorescence in situ hybridization. Since neither a metastasis nor a residual tumor was found, no further adjuvant therapy was needed. Results: We searched the literature for previous case reports and identified only 12 cases worldwide to form our database. A demographic as well as clinical and histopathological analysis was made. In most cases, the liposarcoma occurred in patients >60 years of age. All histological subtypes, such as well-differentiated and myxoid liposarcomas, and pleomorphic and dedifferentiated liposarcomas, were found in the literature. In only 38.46% of the cases, an infiltration of the adjacent organs was observed. Surgery was the most common treatment chosen. Conclusions: Our review provides clinical and histopathological features of a primary liposarcoma of the thyroid to enable the identification of this rare tumor entity and assist in the decision-making process regarding therapeutic options and tumor follow-up.