We herein report a patient who clinically presented with a pigmented, flat plaque in the vulvar area. Histological examination showed a benign lesion mainly composed of tubular and cystic glands with apocrine differentiation. The most striking histological feature was the deposition of finely granular melanin pigment both in the epithelial cells and in the luminal surface of the glands. In addition, Melan-A immunostaining showed the presence of numerous melanocytes within the lesion suggesting that the pigment deposition was secondary to colonization of the lesion by melanocytes. We therefore diagnosed this lesion as "pigmented apocrine hamartoma." To the best of our knowledge only 3 cases of pigmented apocrine hamartoma have been reported in the literature so far.
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