Severe Pulmonary Stenosis as Vascular Manifestation of Von recklinghausen disease in adultsNeurofibromatosis type 1 (NF1), also known as Von Recklinghausen disease, is the most common neuro-cutaneous syndrome estimated to occur in approxi-mately 1 out of every 3,300 infants. Despite the most frequent clinical manifestations are café au lait spots and cutaneous neurofibromas, the presence of multi-system involvement may lead to diverse clinical mani-festations. (1, 2)The cardiovascular manifestations include systemic hypertension and pulmonary artery hypertension, ste-nosis of the renal artery, congenital heart defects, hy-pertrophic cardiomyopathy and less frequently pheo-chromocytoma. In the National Neurofibromatosis Foundation International Database, among 2,322 pa-tients with definite NF1, 2.3% presented cardiovascular abnormalities. Class II flow defects are the most com-mon defects in the great vessels, and include pulmonary valve stenosis, pulmonary subvalvular stenosis and co-arctation of the aorta. (3, 4) The diagnosis is commonly made during childhood and in young adults. (3-5)We present a 71 year-old female patient who sought medical care due to class II-III dyspnea. At physical examination, an intense systolic murmur was heard at the pulmonic area accompanied by thrill. A particularly striking feature was the presence of large, soft, brownish-colored tumors on the face and trunk (Figure 1).Two-dimensional echocardiography and Doppler ultrasound demonstrated the presence of severe pul-monary valve stenosis with a 100 mmHg gradient, mild subvalvular gradient in end-systole and mild dilation of the right chambers. Such findings corre-lated with those seen at cardiac magnetic resonance imaging, providing key information about the absence of other heart defects associated with the underlying disease (Figure 2).The biopsy of a skin lesion showed well-circum-scribed lesions composed of spindle-shaped cells with myxoid collagenized stroma, a typical finding of the disease. (5)