Squamous cell carcinoma (SCC) is the most common tumour of the penis and prepuce of the horse and is found mainly in older animals. Reports on breed predilection for penile tumour formation are equivocal but castration, coat colour, poor genital hygiene and various infectious agents have been suggested to predispose to the development of SCC. Careful assessment of the primary tumour is an important first step in the design of an optimal treatment protocol. Tumour size, invasiveness, differentiation grade and presence of metastases are relevant to the decision to pursue additional diagnostic procedures or specific treatment options. Complete elimination of the neoplasm and, therefore, risk of recurrence is highly dependent on the type of therapy chosen. However, the size and histopathological features of the primary tumour are also important factors with respect to prognosis. This review describes the clinical and pathological features of penile and preputial SCC in the horse and proposes a standard staging system.