Summary: This review aims at giving an overview of the aetiology, genetic basis and clinical signs of equine malignant melanoma (EMM), the diagnostic work-up and the current treatment options. The established association between certain genetic features and melanoma formation in horses is reviewed. Typically, grey horses are affected with EMM. Typical predilection sites are the ventral tail, perianal region, eyelids, lips, parotid, or guttural pouches. Tumours usually grow slowly, but frequently metastasize eventually. Chemotherapy with cisplatin, 5-Fluorouracil and cimetidine are reviewed. Surgical excision of solitary small tumours can be successful, but has no systemic therapeutic effect and cannot prevent tumour recurrence. In conclusion, therapeutic options are still limited and conventional therapy is hardly curative. Current research focuses on immune modifying therapies using either tumour antigens for therapeutic vaccination or antitumoural interleukins. The most promising therapeutic approach currently under investigation is gene therapy using a xenogenic DNA vaccine coding for tumour associated antigens such as tyrosinase and gene therapy using interleukin-12 and -18.