Introduction Necrotizing Fasciitis (NF) is a rare, rapidly progressive, and potentially fatal infection that mainly affects the subcutaneous tissue and fascia. Vulvar NF is an extremely rare presentation with an incidence still unknown, it presents a mortality rate in women of up to 50%. Clinical case 39-year-old female patient with morbid obesity, insulin-requiring type 2 diabetes, high blood pressure and hypertriglyceridemia. Presents increased volume in the right labia majora of 1 week of evolution with an increase of pain, local heat, and erythema. Without fever, general involvement, and purulent discharge. The patient evolves with septic shock and vulvar NF with extension to the right groin without involvement of the vagina, urethra, and anus. A radical vulvectomy was performed, antibiotic therapy was completed, and closure and vulvoplasty were performed in the second surgical stage. Discussion Vulvar NF is especially challenging due to the anatomy and functional structures that can be affected by the infection. Two microorganisms Gleimia europaea and Enterococcus were found. This case demonstrates how early and aggressive surgical intervention associated with antibiotic therapy can be of vital importance to reduce mortality, complications, and patient’s sexual, urination and defecatory health to remain intact.