The presence of the single lung lesion with only one lymphadenopathy paratracheal with pathological and immunohistochemical (IHC) profile similar to the vulvar lesion, and a particular IHC profile with CK7+ and CK20- was detected - that is more specific to the primitive pulmonary cancer, and the presence of only one sarcoma marker vementine+, desmine and actine-. Also the presence of KI 67: 20%, predicted the proliferative and great metastatic power of the lung tumor was observed. Additionally, lung cancer was the most frequent type and may develop in scleroderma as reported in most studies. This allows to conclude for primitive lung carcinoma revealed with vulvar metastasis after elimination of the possibility of vulvar sarcoma. The patient was treated by chemotherapy (Taxol/Platin) with partial response from the lung after 3 cycles and palliative radiotherapy in the vulva with a good response. This case described primary lung carcinoma associated with scleroderma, revealed by a vulvar metastasis, which may be related to the aggressiveness of lung cancer when the lung fibrosis follow-up is not performed well to detect early the development of lung tumors in the patient with systemic scleroderma.