Abstract
Epithelioid sarcoma (ES) is an uncommon soft tissue sarcoma. It is usually located in the extremities and exceptionally in the neck. Its diagnosis constitutes a real challenge which is based on histology and immunohistochemistry staining that must be interpreted with caution given the anatomopathological similarities to other tumors. In this article, we report a case of a 37-year-old man admitted for a locally advanced ES of the neck. There were suspicions of lymph node metastases of nasopharyngeal carcinoma at the first pathological examination. The patient received palliative chemotherapy and was referred to the supportive care department. Through this case, we will discuss the clinical and anatomopathological characteristics and therapeutic options of this extremely rare tumor which poses a diagnostic challenge.