We report a case of arterial fibrodysplasia causing vascular disease with ulceronecrotic lesions in lower extremities. The arteriography showed a beaded pattern in both arteries and veins; laboratory test findings were within normal value ranges; the biopsy reported medial hyperplasia involving the arterial and venous walls. Arterial fibrodysplasia should be considered as a differential diagnosis in patients complaining of recurrent occlusive vascular episodes without systemic manifestations, normal laboratory test results, and radiological patterns suggestive of fibrodysplasia. This is the first report of medial hyperplasia in digital arteries and venous wall involvement.