Syringoid eccrine carcinoma is a very rare skin cancer. We present a case of a 22-year-old woman with a presentation of syringoid eccrine carcinoma in the subungual region of the hallux. This clinical case demonstrates our work-up that led to a proper diagnosis and management of this pathology. We discuss our surgical procedure of choice and the outcome. This report adds valuable information to a limited database of knowledge available on the diagnosis and management of syringoid eccrine carcinomas.