Myofibroblastic sarcoma can be classified as low-, intermediate-, and high-grade. Low-grade myofibroblastic sarcoma (LGMS) is uncommon and rarely appears on the skin. LGMS is diagnosed based on histopathological and immunohistochemical findings. Additionally, LGMS metastases are rare as well. Herein, the authors describe a case of cutaneous LGMS in the neck. To the best of our knowledge, there are no cases regarding cutaneous LGMS in the neck present in the existing literature. Surgical resection is considered as the most important treatment for LGMS. Therefore, a complete tumor resection was performed in this case. However, another mass in the uterus was identified on abdominal computed tomography performed post-surgery.