Rhabdomyosarcoma affects mainly pediatric patients and is currently classified into 4 categories: embryonal, alveolar, pleomorphic, and spindle cell/sclerosing. Epithelioid rhabdomyosarcoma is a recently described variant of rhabdomyosarcoma in which primary cutaneous presentation is infrequent. In this brief report, we describe a rare case of epithelioid rhabdomyosarcoma in an 81-year-old man, presenting as a skin lesion in the neck, which increased in size in 1 month. After imaging evaluation, a solid cervical mass was discovered. A biopsy was performed, and the diagnosis of epithelioid rhabdomyosarcoma was rendered. The patient died due to rapid progression of the tumor. To make an accurate diagnosis and ensure appropriate patient management, it is necessary to be aware of this variant and use proper immunohistochemical stains when facing an epithelioid malignancy, expanding the differential diagnosis of epithelioid neoplasms.