Non-squamous cancers of the vulva encompass an exciting and broad group of tumours, including Bartholin's gland carcinoma, malignant melanoma, Paget's disease, sarcomas and lymphoma. These tumours range from innocuous lesions treatable with simple local excision, such as basal-cell carcinoma, to cancers with very poor prognosis, such as Merkel-cell tumours. All of these tumours are thoroughly reviewed, with emphasis on presenting symptoms, pathological diagnosis and optimal management approaches. The literature supporting these recommendations is reviewed. Of the utmost importance in the management of these tumours is a thorough review of the pathological diagnosis by a specialist pathologist and a gynaecological oncologist. Establishing the correct diagnosis is essential to reaching appropriate treatment decisions. Frequently this will necessitate a second opinion by a referral centre.