This report describes the successful treatment of a cutaneous hemangiosarcoma of a 12 years old horse presented for evaluation of a cutaneous mass on the left gluteal region. Hemangiosarcoma is a rare life-threatening neoplasia that can be successfully managed when diagnosed and treated at early stages. Unfortunately, they are often diagnosed at later stages, leading to a poor outcome and low survival rates. In this report, the horse has been treated with a complete large surgical excision of the neoplastic tissue. A good cosmetic outcome as well as a short recovery time has been achieved with an autologous skin grafting split thickness modified meek technique. A long-term follow-up of the patient showed no reoccurrence of the hemangiosarcoma, and successful return to athletic performance. In the authors knowledge, this is the first equine primary cutaneous hemangiosarcoma treated with complete excision and subsequently autologous skin grafting with a long-term follow-up and good outcome despite the poor prognosis.