About 7 % of all soft-tissue sarcoma are leiomyosarcomas (LMS). The primary tumor is usually found in the uterus, the gastrointestinal tract, retroperitoneum and extremities. The most common site of metastases of LMS is the uterus but they are also found in the liver, lungs, kidney, skin, bone and the brain. Brain metastases may become symptomatic with multiple hemorrhages [4]. Only 1 % of them derive from soft tissue sarcomas, a fifth of them being LMS [2]. Spinal metastatic manifestations are rare. They usually arise from vertebral bone lesions and give rise to compression fractures or expand as extradural space occupying lesions into the spinal canal with subsequent spinal cord compression [1, 3, 5, 6]. We present the first case of a neurologically symptomatic, intramedullary spinal LMS metastasis without vertebral bone lesions (Fig. 1). LMS metastasis was histologically diagnosed (Fig. 2) following spinal cord biopsy. It was secondary to a surgically removed gastrointestinal LMS 13 years before. The 76-year-old male, immune-competent patient suffered from a 2 months history of progressive weakness and numbness in his legs and gait unsteadiness. On examination there was spastic paraparesis (MRC 2–3) with increased tendon reflexes in the legs, a sensory level (Th12, L1) of hypaesthesia, hypalgesia and severely reduced position sense and partial urinary incontinence. Past medical history was significant for a surgical resection of an oesophagus tumor (pT1, pN0) and adjacent lymph nodes in 1998 which histologically was diagnosed as LMS based on typical mitotic behaviour and Actin-, Vimentinand Desmin-positive immunoreactivity. Five years later he developed abdominal pain attacks which were caused by metastasis of the previously diagnosed LMS in the small intestine which was surgically removed. Subsequently, he was asymptomatic until 2011 when he developed myelopathic signs. Spinal MRI showed a round-shaped lesion in the caudal thoracic spinal cord at the level of vertebra D12 suggesting an intramedullary tumor (Fig. 1). The lesion could be clearly delineated from the surrounding intact spinal cord with oedema at its caudal and cranial pole (Fig. 1a). The lesion homogeneously enhanced after gadolinium application (Fig. 1b). It expanded almost throughout the transverse spinal cord leaving only a small rim of unaffected superficial tissue. Shape and size of the tumor were found not to be altered on follow-up MRI 3 weeks later. Noticeably, there were no bone lesions in the adjacent vertebral bodies. Cranial MRI and CCT did not reveal additional lesions. Bone scintigraphy also C. Helmchen A. Geaid Department of Neurology, University Hospitals Schleswig-Holstein, Luebeck, Germany