A case of myofibrosarcoma (IMT) of the brain and lung as well as the spinal cord is described. A 29-year-old male patient presented with fever (40 degrees C), malaise, vomitus, meningism and leukocytosis. Computer tomography identified a bleeding in the left frontal lobe. A bleeding angioma was suspected and an operation was performed. The histological examination could not reveal an exact diagnosis. Eight months after complete recovery from the first bleeding, the patient had a second intracranial temporo-occipital bleeding on the right side which has been removed operatively. A new lesion was seen in the left parietal white matter of the brain. A growing cavernoma was suspected and resection of the lesion was planned. Pre-operatively the patient suffered from hemoptysis and fever. The X-ray of the chest showed a pulmonary lesion in the left lower lobe. In the CT of the chest a large tumor in the left lower lobe of the lung and additionally a cystic structure in the mediastinum was seen. The histological examination of this tumor identified an inflammatory myofibroblastic tumor (IMT). The left parietal lesion has been resected after the thoracic operation. The brain lesions were estimated to be metastases of the IMT of the lung. In the further clinical history the patient developed a large spinal cord metastasis of the thoracic spine. The metastatic development of the tumor reported in this case is unusual. The current therapy of these tumors consists of complete tumor resection and further clinical controls. However, due to the localization and the extension of some lesions in the present case, the complete resection has not been possible. There is no proven role of chemotherapy and radiation therapy. The patient died due to the pulmonary deterioration.