Introduction: Neuroblastoma is a neoplasm arising from the primordial neural crest cells that form the sympathetic nervous system. Orbital neuroblastoma is typically a metastatic tumor. Accounting for 10–15% of childhood tumors, neuroblastoma is the second most common orbital tumor in children, after rhabdomyosarcoma.Neuroblastoma can also develop anywhere along the sympathetic nervous system chain from the neck to the pelvis. Neuroblastoma occurs primarily in the abdomen in 60% cases but in 8% cases the tumour arises in the orbit where it arises from ciliary ganglion. Toreport a rare case of neuroblastoma with ocular proptosis as an initial presentation.

Case Report: A case of seven months old baby who presented with rapidly progressing proptosis within three months. A team of multidisciplinary specialists was conducted to manage the case. Computerized Tomography Scanner (CT Scan) showed visible heterogeneous lesions in retrobulbar region, extends from superior orbital fissure to optical chiasm with destruction of the surrounding bone. Histopathologic diagnosis of neuroblastoma was made. Subsequent medical evaluation including laboratory, chest X-ray, and whole body computed tomography showed no evidence of systemic involvement or metastasis. The child was subjected to chemotherapy, but due to late presentation the patient deceased after the first cycle of chemotherapy.

Conclusion: Neuroblastoma should be considered in the differential diagnosis of rapidly progressing proptosis in infant. Care must be taken to manage the case aggressively as delayed diagnosis and treatment in rapidly growing tumor would have led to death.

Keywords: Childhood orbital tumors, Neuroblastoma, Primary orbital neuroblastoma