A Chiari malformation Type I may remain asymptomatic until the patient has reached adulthood and acute presentation of symptoms occurs. In several clinical and experimental studies it has been shown that essential hypertension is associated with vascular compression of the brainstem, particularly of the rostral ventrolateral medulla oblongata. Nevertheless, two cases of Chiari malformation and neurogenic arterial hypertension have been reported. In this article the authors describe a patient with Chiari malformation Type I and neurogenic arterial hypertension. A simple suboccipital decompression not only provided neurological improvement, but also led to resolution of the hypertension. In cases of Chiari malformation and concomitant neurogenic arterial hypertension, careful preoperative clinical and neuroimaging assessments may reveal the cause of the arterial hypertension. Resolution of neurogenic arterial hypertension may be expected even in a case of simple suboccipital decompression.