Introduction/Background Mucoepidermoid Carcinoma (MEC) is an epithelial malignant tumor that was first described as a salivary gland malignancy. Though common in salivary gland, it is extremely rare in the vulva with only 2 cases reported in the English language literature and none yet in the Philippines. The objectives of this case report are to present an extremely rare case of MEC in the vulva and describe its clinico-pathologic characteristics, explain how MEC may occur in this unusual location and discuss its management and other treatment options.

Methodology This is a case of a 68 year-old woman with a history of vulvar pruritus and vulvar mass at the left labia majora. Punch biopsy and review of slides revealed Invasive Squamous Cell Carcinoma, Non-Keratinizing type. She underwent Radical Vulvectomy and Bilateral Lymph Groin Dissection; Wide Excision of Perineal Area; Protective Transverse Loop Colostomy; Gracilis Myocutaneous Flap with Identification of Right and Left Median Circumflex Artery with a final histopathology report of Primary Cutaneous MEC of the vulva with lymph node metastasis.

Results Due to its low incidence, prognosis and definitive management is still unclear. The patient was started on adjuvant chemotherapy, followed by Intensity Modulated Radiation Therapy, as in other histopathologic diagnosis of advanced stage vulvar carcinoma.

Conclusion This is a case of a primary cutaneous MEC of the vulva in a 68 year-old woman who presented with a history of intermittent vulvar pruritus and vulvar mass. To the best of our knowledge, this is the first reported case of vulvar carcinoma having this rare pathology in our local setting. Due to limited studies available, tailored management should still be investigated.

Disclosure Nothing to disclose