Aim: The aim of this study was to present a rare benign tumour of neural origin located in the orbit.
Material and methods: Authors describe case of six-year-old boy with tumour of the right orbit, upper eyelid and right zygomatic fossa. Medical history revealed gradually increasing in size, painless tumour. Before admission to the hospital computed tomography was taken to assess the size, localization of the tumour and to plan the proper treatment.
Results: The patient was operated on using Krönlein approach. Tumour was removed in macroscopically radical limits without need to incise the muscles responsible for eye movement. Histopathological examination revealed: Neurothekeoma, classic type. During one year follow-up there was no recurrence.
Conclusion: Neurothekeoma may occur in young boys in eyelid and orbit. A unique localization of neurothekeoma in the orbit, close to the lacrimal gland shows that this kind of histopatological tumours may infiltrate deeper tissues. Surgical treatment of neurothekeoma is the same as in other benign tumours.