Introduction: Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type I. Orbital neurofibromas are relatively rare and usually present with high infiltration into the periocular structures making the management extremely difficult. Purpose: To r eport a c ase o f p lexiform n eurofibroma w ith neurofibromatosis type I in a young boy managed satisfactorily with surgical excision. Materials and methods: A 14-year-old boy presenting with a soft, painless, nontender, gradually enlarging swelling of the left upper eyelid and adjacent temporal region and of 6 years duration was managed with complete surgical excision. Histopathological examination of excised mass confirmed the diagnosis of neurofibroma. Results: Satisfactory functional and cosmetic result was achieved following surgical excision of the subcutaneous fibromatous mass. A 1-year follow-up did not reveal any functional deficit or recurrence. Conclusion: Plexiform neurofibroma may present as an uncommon cause of periorbital swelling even in the absence of typical features of neurofibromatosis type I and can be surgically managed to achieve acceptable cosmetic and functional results.