Epithelioid haemangioendothelioma (EHE) is an uncommon type of vascular tumour with intermediate malignant potential, classified as a sarcoma which occasionally involves neurovascular structures and can mimic nerve sheath tumours. EHE is difficult to distinguish from other nerve sheath tumours based on imaging, including MRI, and biopsy is often required for diagnosis. Diagnosis of EHE from biopsy often requires the use of vascular immunohistochemical stains. We present a case of left upper limb neurovascular bundle EHE presenting with proximal ulnar nerve neuropathy and subsequent median nerve neuropathy and liver, lungs and bone metastases. The tumour had been identified 20 years prior with a similar presentation of upper limb weakness and sensory disturbance, yet following surgical excision it was misdiagnosed as inflammatory fibrous tissue. Treatment with propranolol has resulted in disease stability and surgical debulking resulted in improved upper limb function. The use of beta-adrenergic receptor antagonists in EHE and other sarcomas have been shown to increase T-cell infiltration and decrease immunosuppressive PD-1 expression in neoplastic cells.