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Background: We describe 2 cases of vulvar pseudotumour due to lymphatic obstruction with chronic lymphoedema of unknown cause that presented as a solitary mass that mimicked aggressive angiomyxoma. Material and Methods: Both patients presented with a vulvar mass without medical history of trauma, surgery in the anogenital region or skin diseases. One patient was overweight (BMI = 26). Both surgically resected vulvar specimens were represented by a polypoid mass with a soft and a gelatinous cut surface. Results: Histologically, the presence of an abundant oedematous stroma with spindle-shaped cells and numerous thin-walled small-to-medium vessels may be confused with an aggressive angiomyxoma. The diagnostic key was represented by the massive oedema, rather than myxoid stroma, with the presence of dilated, tortuous lymphatic channels (some surrounded by clusters of lymphocytes) in the dermis. Conclusion: The recognition of these lesions is important because they may be the cause of problems in differential diagnosis and therapeutic management. In fact, such lesions can be mistaken from both the clinical and histological perspective as a primitive tumour of the vulva-like aggressive angiomyxoma. However, these lesions are not true neoplasms and are likely due to lymphatic obstruction with lymphoedema. A simple surgical excision with vulvoplasty is curative. Copyright © 2010 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]