This is the first case report for the positive Trypanosoma evansi incident in Kelantan, Malaysia confirmed through protozoa detection in a Siam B mare. The horse was presented with complaints of lethargy and inappetence and it collapsed on the day of visit. Normal saline and dextrose solution were administered intravenously, while multivitamins and nerve supplements were given intramuscularly to stabilise the horse before further treatment. Haematological findings showed normocytic hypochromic anaemia and are suggestive of regenerative anaemia. Thin blood smear and examination revealed the presence of Trypanosoma sp., and it was confirmed as T. evansi through molecular identification. The horse was found dead 2 days after and post-mortem was conducted. Histopathology revealed that the horse had developed a neurological form of the disease, besides the detection of the protozoa in heart, spleen and kidney tissue. This first positive Surra case in Kelantan, Malaysia, that is bordering Thailand confirms the increasing concern of transboundary infections. In conclusion, Surra is a potential emerging disease and should be considered as differential diagnosis in horses with pale mucous membrane. This condition is particularly imperative in horses found in these regions as Surra is endemic.