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Cutaneous angiosarcoma is a type of rare and locally aggressive malignancy requiring individualised treatment owing to paucity of randomised trials. We present the case of a middle-aged cancer survivor with locally advanced angiosarcoma of scalp managed with surgery, radiotherapy, chemotherapy and targeted therapy over a course of 6 years for two recurrences. The first recurrence was preceded by opsoclonus myoclonus syndrome, a type of paraneoplastic neurological syndrome (PNS), rarely reported in sarcomas. The second recurrence had a rapi