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The authors report a rare clinical case of a patient with neurofibromatosis type 1 (NF1) complicated by pulmonary hypertension (PH), which presents with rapid progression. An exhaustive investigation was performed to identify the main aetiology of the PH. It was concluded that the PH could be associated with NF1, and so belonged in group 5 of the clinical classification of PH. In general, such patients have a poor long-term prognosis due to the inexistence of proven, effective treatment. Further studies are needed to better understand the mechanisms of NF1-associated PH.

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