Kim, Usha RArora, VipulDevanand, JKhazei, Hadi M2011-12-202011-12-202009-09Kim Usha R, Arora Vipul, Devanand J, Khazei Hadi M. Multimodality treatment approach in management of primary peripheral primitive neuroectodermal tumor of the orbit. Indian Journal of Ophthalmology. 2009 Sept; 57(5): 395-398.http://imsear.searo.who.int/handle/123456789/135987Primitive neuroectodermal tumor is a small round cell malignancy which rarely involves the orbit. We report a case of a two-year old male child presenting as unilateral eccentric proptosis with extraconal and intraconal mass, diagnosed as primary peripheral primitive neuroectodermal tumor (pPPNET) on histopathology and immunohistochemistry. There is no defined consensus in the management of these tumors due to its rare presentation. We describe its distinguishing features with emphasis on multimodal and aggressive treatment approach which ensures appropriate management of these cases.enExtraosseous Ewing's sarcomanonspecific enolaseprimitive neuroectodermal tumorAntineoplastic Agents --therapeutic useBiopsyChild, PreschoolFollow-Up StudiesHumansMagnetic Resonance ImagingMaleNeuroectodermal Tumors, Primitive, Peripheral --diagnosisNeuroectodermal Tumors, Primitive, Peripheral --drug therapyNeuroectodermal Tumors, Primitive, Peripheral --radiotherapyOrbital Neoplasms --diagnosisOrbital Neoplasms --drug therapyOrbital Neoplasms --radiotherapyRadiotherapy, AdjuvantTomography, X-Ray ComputedArticle