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INTRODUCTION: Vulvar cancer accounts for approximately 5% of all female genital malignancies and is commonly recognized as a disease of postmenopausal women. The vast majority of vulvar carcinomas are of the squamous cell type (90%) with primary vulvar adenocarcinomas ranking among the rare gynecological malignancies, comprising less than 0.1% of the cases. This case report is a primary vulvar adenocarcinoma and discusses the diagnosis and the treatment of this unusual and uncommon neoplasm. METHODS: The clinical case was analyzed followed by a literature review on papillary adenocarcinoma of the vulvar. RESULTS: A 53-year-old postmenopausal African-American female presented with 1-year history of enlarging, otherwise asymptomatic left vulvar mass. A flesh colored 3 × 2 cm mobile, nodular lesion was noted on left anterior labia majora. An excisional procedure was completed. Final pathology revealed the skin to be benign with cyst wall and contents revealing moderately differentiated invasive papillary adenocarcinoma. Stains of the neoplastic cells were positive for p53 immunostain. Follow-up clinical evaluation did not demonstrate evidence of metastatic disease and the recommendations of the monthly multidisciplinary meeting was for ipsilateral lymph node dissection and modified radical vulvectomy of tumor bed. The patient declined diagnosis and recommended treatment. CONCLUSION: Adenocarcinomas of the vulva are exceedingly rare, making diagnosis and management difficult for pathologists and clinicians. As a result literature is very limited. This report will allow for further advancement in regards to the diagnosis and treatment of this unusual and uncommon neoplasm.