A 55 year old man was admitted to hospital with a head injury that resulted in hearing loss on the left side with pulsatile tinnitus and a vague feeling of disorientation. A CT scan showed an irregular extra-axial solitary lesion lying adjacent to the falx on the right of the midline measuring 3 × 4 × 6 cm with significant mass-effect and peritumoral oedema involving most of the right frontal lobe. There was evidence of underlying bone remodelling with vascular dural supply and heterogeneous enhancement after contrast administration with a deep cystic component. There were fringes of tumour interdigitating with the brain substance indicating probable brain invasion. The findings were consistent with an aggressive frontal meningioma. Partial surgical removal of the lesion in the right frontal lobe was undertaken. On histological examination, the lesion was classified as an atypical (WHO Grade II) meningotheliomatous meningioma. A year later, the patient experienced an episode of seizures and a CT scan revealed three new lesions at the site of the previously excised meningioma with extensive perilesional oedema. An MRI confirmed the CT findings indicative of tumour and also detected invasion and occlusion of the anterior part of the superior sagittal sinus. Partial resection of the recurrent mass was again undertaken. Histology of the tumour specimen showed features of an anaplastic (WHO Grade III) meningotheliomatous meningioma with lobules of tumour containing cells with round or oval vesicular nuclei. Focally, there were cells which showed more prominent nuclear pleomorphism. There was increased mitotic activity and extensive tumour necrosis was noted. Post-operative follow-up revealed residual tumour in the anterior part of the superior sagittal sinus, along the falx and the right frontal convexity. Follow-up imaging also confirmed interval progression of the residual meningioma. The patient completed a course of radiotherapy and, nine months later, a mobile soft tissue lump on his back was noted. A thoracic CT scan revealed a soft tissue lesion superficially located in the inferior portion of the left trapezius muscle. The lesion was lobulated, had a low attenuation centre, showed peripheral enhancement and did not exhibit evidence of matrix calcification. The lesion increased in size and a subsequent MRI scan performed 6 months later confirmed the presence of a solid soft tissue mass up to 5 cm in diameter in the medial left trapezius muscle. The lesion indented the underlying paravertebral muscles but remained well-defined and did not invade the deeper musculature. The lesion returned isointense T1-W signal relative to skeletal muscle and heterogeneous high T2-W signal. There was no internal calcification, haemorrhage or cystic degeneration. Peripheral feeding vessels were present. A PET-CT scan demonstrated that the intramuscular lesion was extremely FDG avid with an SUVmax of 22.1. PET-CT also revealed disseminated, markedly FDG avid metastases in liver, bone and pleura and residual meningioma in the right frontal lobe. The lesion was biopsied and showed a largely lobulated tumour containing collections of tumour cells, many of which showed similar features to those seen in the frontal lobe tumour. There were a few whorled collections of tumour cells with round or oval vesicular nuclei. There was prominent nuclear pleomorphism, a high mitotic rate and focal tumour necrosis. Focally there were solid areas of proliferation of pleomorphic tumour cells, some of which had vacuolated cytoplasm. Immunohistochemistry showed strong expression of epithelial membrane antigen (EMA) and P63; there was also focal expression of cytokeratin (CK7+, CK20−), nuclear staining for IN-1, and a high Ki-67 fraction. There was no expression of desmin, S100, CD10, CD30, CD68, myogenin, smooth muscle actin, estrogen receptor, progesterone receptor, chromogranin, carcinoembryonic antigen or TTF1. There was initially some difficulty in establishing the pathological diagnosis as the previous history of recurrent atypical/anaplastic meningioma was not provided to the reporting pathologist whose initial differential diagnosis included a soft tissue metastasis of carcinoma and a primary soft tissue sarcoma expressing the epithelial markers. However, once the history of meningioma and the radiological findings were taken into account, it was clear that the soft tissue mass represented a metastasis of the previously diagnosed anaplastic meningotheliomatous meningioma. A wide local excision of the lesion, which measured 5 × 3 × 2 cm, was performed; this included a cuff of normal muscle around the lesion and a 1 cm margin of skin around the previous biopsy scar. Histological findings in the resection specimen were similar to those of the biopsy. Further follow up of the patient by CT scan showed no recurrence of the soft tissue lesion but progression of disease in the brain.