ABSTRACT
Papillary hidradenoma of the vulva is a rare, benign neoplasm arising from apocrine sweat glands of the skin. Frequently, this lesion has been mistaken for carcinoma. The treatment of choice is local excision. The prognosis for patients with this tumor is excellent. We present a patient who is unique because she had a lesion which was the largest ever recorded, and which existed over twice as long as any previously described. This case is also presented to remind the clinician that, despite the gross appearance of the tumor which resembles carcinoma on sectioning, biopsy and histological diagnosis should guide the ultimate management of patients with such lesions. The findings in our patient support the view that no matter how large or how long in existence, hidradenoma remains benign.