Objective: The study aimed to report the case of a patient with a polypoid vulvar lesion clinically thought to be an aggressive angiomyxoma.
Materials and Methods: On examination, a 4.0 × 2.0 × 1.5-cm polypoid lesion was seen on the right labium majus.
Results: Histopathological findings of the excised mass were consistent with pseudoangiomatous stromal hyperplasia of the vulva
Conclusions: Pseudoangiomatous stromal hyperplasia is a lesion of breast tissue but can occur in anogenital mammary-like glands. It must be distinguished from low-grade angiosarcoma.