A twenty-four-year-old mare, which had been examined seven years earlier for mitral valve regurgitation and mild left sided cardiomegaly, was presented with tachycardia, profuse sweating and muscle fasciculations. Blood examination revealed an increased packed cell volume, metabolic acidosis, hypocalcemia, hyperglycemia and increased cardiac troponin I concentration. ECG revealed ventricular premature beats and monomorphic ventricular tachycardia followed by polymorphic ventricular tachycardia with R-on-T phenomenon. The horse was treated immediately with hypertonic solution followed by isotonic solution and calcium, but the general condition deteriorated within forty-five minutes after arrival. The horse was euthanized due to poor prognosis. On necropsy, a pheochromocytoma of the left adrenal gland was found. Although this horse had undergone a cardiovascular examination seven and one year prior to the onset of the clinical signs, no indications for a neoplastic process or symptoms of a pheochromocytoma were found at that time. Early diagnosis of pheochromocytoma is based on catecholamine mediated cardiovascular effects, blood examination, blood pressure measurement, rectal palpation and rectal ultrasound of the adrenal gland. Based on a retrospective analysis of echocardiographic images and measurements, no predisposing factors were found. In this article, the importance of including pheochromocytoma as a differential diagnosis is highlighted, especially in older horses with acute polymorphic ventricular tachycardia, sweating, muscle tremors and signs of acute abdominal pain.