Sweat gland carcinoma of the vulva is rare and may be classified as being of eccrine, apocrine, or mixed origin. Most reported cases of vulvar sweat gland carcinomas associated with extramammary Paget disease describe a tumor of apocrine origin. We report a case of a vulvar sweat gland carcinoma of eccrine origin associated with Pagetoid extension. A review of the literature and the differential diagnosis are also presented. To our knowledge, this is the second case of vulvar sweat gland carcinoma of eccrine origin associated with extramammary Paget disease.