Dermatofibrosarcoma protuberans is a malignant tumor of the soft tissue which is characterized by local recurrence with an exceptional metastasize, and for this reason, patients with dermatofibrosarcoma protuberans require a long-term follow-up. This clinical case presents a 41-year-old patient, with no pathological history, who has a malignant tumor of the chest wall, with a pulmonary nodule, which appears on the chest computed tomography scan like a well-rounded parenchymal nodule measuring 15 mm in diameter. This case was discussed in the multidisciplinary meeting which concluded that it is a dermatofibrosarcoma protuberans with metastasis of the lung. In the context of these data, it is thought that the pulmonary nodule is of metastatic origin according to the decision of the multidisciplinary meeting, while the final diagnosis was a hamartochondroma, which is a benign tumor of the lung. The interest of this clinical case is to discuss the possible diagnoses of the pulmonary nodule in the context of malignant wall tumor.