A patient with renovascular hypertension and horseshoe kidney is presented. Diagnostic work-up consisted of ultrasonography, color-Doppler of renal arteries, dynamic scintigraphy with captopril test, separate renin, intravenous urography and computed tomography. Renal angiography revealed several aberrant renal arteries with significant fibromuscular dysplasia of the proximal artery of the right kidney. Contrast magnetic resonance of the kidneys with gadolinium was also performed. Following diagnostic procedure, a 5 mm balloon dilatation of the right proximal renal artery was performed. Immediate renal angiography showed no stenosis of the right proximal artery. The patient's blood pressure normalized after the invasive procedure. The diagnosis and treatment in the patient with renovascular hypertension due to fibromuscular dysplasia and horseshoe kidney are described. As renovascular hypertension is a rare diagnosis, this case report may help physicans recognize patients with this type of arterial hypertension.