Equine sarcoid is the most common tumour of horses and accounts for over half of all equine skin tumours. Six types of sarcoid based on gross appearance and clinical behaviour have been described including occult, verrucous, nodular, fibroblastic, mixed and malevolent. Common locations for sarcoid development include the periocular region, ear pinnae, lips, neck, extremities and ventrum (including groin region). Bovine papillomavirus (BPV) is causally associated with equine sarcoid with genetic haplotype, fly vectors and skin trauma identified as potential risk factors for development of the disease. Histopathology is required for definitive diagnosis of equine sarcoid but incomplete excision is thought to activate latent BPV and stimulate growth. Although there are no uniformly effective treatment options, several modalities have been successful in eliminating or managing equine sarcoid. Surgical excision, intratumoural chemotherapy, cryotherapy, hyperthermia, radiotherapy, immunotherapy and immune modulators are used with degrees of success relative to the accessibility and invasiveness of the tumour. Prevention of equine sarcoid may be facilitated by future development of vaccines against bovine papillomavirus.