In neoplastic processes in the orbital area, a not significant part of these new entities is caused by tumours of the peripheral nervous system. Due to a clinically similar behaviour and radiological features also being similar, these tumours sometimes cause differential diagnostic difficulties. Our experience with these tumours shows that they do not have a uniform symptom complex. Exophthalmus and displacement of the globe are the most visible symptoms besides pain, motility disturbances, reduced visus and double images. Clinical imaging allows the exact localisation of the tumour and makes it easier to choose the best surgical method. However, imaging does not allow the exact assessment of the entity or enable a decision to be made about the possible malignancy of the tumour. Therefore, a representative biopsy and a histopathological examination are essential. Then, a complete tumour resection should be performed using the most careful incision possible to avoid postoperative complications.