Neurofibromatosis is an inherited disease, having a predilection for tumour formation. [1] These diseases were previously referred to as "phakomatoses" or neurocutaneous syndromes. The clinical spectrum of these diseases usually involves the nervous, locomotor and cutaneous systems. However the involvement of the nervous system is very dangerous, and is often the leading cause of mortality in this condition. It disturbs cell growth in the nervous system which leads to the formation of tumours, either malignant or benign, on nerve tissue. The involvement of the respiratory system in these patients is very rare. We present an interesting case of a patient with neurofibromatosis induced diffuse parenchymal lung disease.