In this review, we focus on rare benign and malignant diseases. It is shown that benign vulvar conditions require thorough examination of other parts of the body, because a relationship with systemic diseases, like cicatricial pemphigoid, pemphigus vulgaris, toxic epidermal necrolysis, infections with parasites and Behçet's syndrome, may be present. Co-operation with medical specialists is warranted. One in every 10 malignancies of the vulva is not a squamous cell carcinoma. Apart from melanoma, several other tumors can occur on the vulva. A correct histological diagnosis is of paramount importance because treatment strategies depend on it.