Vulvar squamous cell carcinoma with sarcoma-like stroma represents an extremely rare histological entity showing the co-existence of both epithelial and mesenchymal features: these tumors, firstly described in the skin by Martin and Stewart in 1935 have been further described in other anatomic sites including oral cavity, larynx, breast, lung and oesophagus. The complexity of the histology, as well as its aggressive clinical behaviour makes the diagnosis and the exploitment of effective therapeutic approaches very difficult, so that no definitive guidelines for treatments are currently available. Here, we describe a case of advanced stage vulvar squamous cell carcinoma with sarcoma-like stroma showing an unfavourable prognosis despite the use of an aggressive multimodal approach. A revision of the currently published cases have been also provided.