Granular cell tumor (GCT, Abrikossoff tumor) is an extremely rare, benign tumor with a neurogenic origin occurring most commonly in the upper aerodigestive tract. GCT of the orbit is a rarity, representing a diagnostic and therapeutic challenge. We report on a 26 year old man suffering from unilateral ocular hypertension and retrobulbar pain. Exophthalmos and disturbance of motility were not noted by the patient. The surgical removal of the GCT, extending into the orbital apex, was performed by an interdisciplinary team using a combination of "midfacial degloving" and LeFort I-osteotomy.