Introduction: Smooth muscle tumors of the vulva are uncommon (0.07% of vulvar neoplasms). Their presentation as giant masses may mimic malignant processes, posing a diagnostic and therapeutic challenge due to the histological similarities between benign variants and leiomyosarcoma (LMS). Case Presentation: A 31-year-old woman presented with a progressively growing vulvar mass of 8 months’ duration, initially diagnosed as a Bartholin cyst. Physical examination revealed a 20 × 20 cm mass involving the right labium majus. Following unsuccessful arterial embolization, the patient developed tumor necrosis and septic shock, requiring emergency surgical excision. Results: Initial histopathological examination reported a malignant mesenchymal neoplasm (grade 2 leiomyosarcoma) based on the presence of coagulative necrosis and a Ki-67 index of 15%. However, review by the Sarcoma Committee and a new immunohistochemical study revealed a Ki-67 index of 2% and absence of severe cytological atypia. The observed necrosis was determined to be ischemic/secondary (following embolization and sepsis) rather than tumor-related, leading to reclassification of the lesion as a vulvar leiomyoma. Conclusions: Secondary necrosis and the Ki-67 proliferation index may act as diagnostic confounders in giant vulvar tumors. This case highlights the critical importance of subspecialist review and long-term clinical follow-up (4 years without recurrence in this patient) to avoid oncologic overtreatment of benign conditions with an aggressive clinical presentation.
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