Ewing\'s sarcoma (ES) is a malignant small round cell neuroectodermal tumour primarily affecting children in the first and second decade of life. Since ES is difficult to control, early diagnosis is crucial, and the treatment requires a multimodality approach constituting chemotherapy, surgery and radiotherapy. Here, we present a case of ES of orbit in a 17-year-old female diagnosed in 2021. This case report focuses on the clinicopathological presentation and management principles of this rare manifestation of the disease that unilaterally infiltrated into the extraconal space of the orbit as well as the I/L frontal sinus. The patient underwent chemotherapy and radiation, and she is on follow-up.

Rare cases of Ewing\'s sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2.2 (NK2 Homeobox 2, a protein coding gene) and subsequently showed EWSR1 rearrangement. The histological feature, immunohistochemical results and genetic fluorescence in situ hybridization analysis of this case were confirming the diagnosis of EWS/PNET. Adjuvant chemotherapy was suggested, but the patient was lost to follow-up.