Abstract
Neuroblastoma, the most common extracranial solid tumor in children, typically originates in the adrenal glands but can occasionally present as primary orbital neuroblastoma. This rare condition, arising from the ciliary ganglion, poses significant diagnostic and therapeutic challenges. We report a case of primary orbital neuroblastoma in a 2.5-year-old female with progressive, painless swelling of the right lower eyelid. Clinical examination showed mild proptosis and a firm lower lid mass. Imaging and histopathology confirmed neuroblastoma, with no other primary sites identified, indicating primary orbital neuroblastoma. The patient responded well to chemotherapy and radiotherapy. A review of the literature revealed only 12 previously reported cases. This malignancy often presents with ocular symptoms and lacks a primary site elsewhere. Diagnosis relies on histopathology and immunohistochemistry. Due to its rarity, there are no standardized treatment protocols, necessitating individualized, multidisciplinary management and long-term follow-up to improve outcomes and understand prognosis.