Metastatic neuroblastoma with opsoclonus raccoon eye presentation is an extremely rare condition presented in stage IV metastatic condition. Histopathology/ cytology, vanillylmandelic acid (VMA), homovanillic acid (HVA) levels and metaiodobenzylguanidine (MIBG) scanning are mainly contributors for the diagnosis of this disease. We are presenting one year male child with history of protrusion of right eye, swelling in right periauricular region, opsoclonus movements of small amplitude and periorbital ecchymosis ‘raccoon eye’. So far only infinitesimal cases has been reported in the literature and our present case report is an extremely rare case of locally advanced metastatic neuroblastoma, who presented as opsoclonus raccoon eye and despite of the aggressive chemotherapy, didn’t responded to the intended treatment. We attempt a review in the literature for different possibilities of staging and diagnostic management.
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