We report the first documented Malaysian case of aggressive angiomyxoma (AAM) of the vulva. A 56-yr-old woman of Indian ethnic origin presented with a vulval lesion which was clinically mistaken for a Bartholin's cyst. The lesion was surgically excised and a diagnosis of AAM was made histologically. Of particular interest was the finding of foamy and mononuclear inflammatory cells and fibrin in the walls of most of the lesional blood vessels. The patient recovered uneventfully and remains without tumor recurrence at the time of writing 37 mths after initial presentation.