This report describes 2 cases of localized vulvar lymphedema that were diagnosed in 2 morbidly obese women, including a unique case of a massive localized vulvar lymphedema associated with lymphangioma circumscriptum of the vulva. Herein, we also review previously reported cases of vulvar lymphedema and discuss differential diagnostic considerations. Our review of the recent literature showed 17 cases that we considered approximately similar, reported under the appellations "vulvar lymphoedematous pseudotumor," "massive vulval edema," "vulvar hypertrophy with lymphedema," and "localized lymphedema (elephantiasis)." The patients ranged in age from 19 to 59 years (average 38.5 yrs), and typically presented with papillomatous plaques, skin polyps, generalized vulvar enlargement, or massive pedunculated masses that had been present for durations that ranged from 3 months to 36 years. The average lesional size was 6.1 cm (range: 0.6 - 45 cm), and 10 (59%) of the 17 cases were 3 cm or less. Excisions were generally curative, although persistent or recurrent disease was reported in 3 cases. The patients were overweight in 9 (75%) of the 12 cases in which the patient weight was noted, and 2 others had chronic immobilization. Stromal edema was the only morphologic finding that was uniformly seen in all 17 cases. However, the following morphologic features were identified in significant subsets: multinucleated giant cells, dermal fibrosis, dermal lymphangiectasia, dermal chronic inflammation, perivascular chronic inflammation (superficial and/or deep), hyperkeratosis, acanthosis, and blood vessels of varying calibers. Several attributes of localized vulvar lymphedema may cause them to closely mimic aggressive angiomyxoma, a differential diagnosis that is herein discussed in detail. Localized vulvar lymphedema may also be a small lesion, and can potentially mimic other myxedematous tumors of the vulvovaginal region. The strongest clinical association is with obesity. The term "localized vulvar lymphedema" is an appropriate generic descriptor for the spectrum of lesions whose fundamental and underlying etiology is thought to be chronic lymphedema.