A 16-year-old male presented with constipation, acute retention of urine and abdominal swelling associated with pain. Per-rectal examination revealed painful growth in lateral and anterior wall of rectum 4cm from anal verge. X-ray erect abdomen was done which showed Indistinct bowel gas pattern suggestive of Intestinal obstruction. Diversion transverse colostomy was done in view of acute intestinal obstruction. Investigations revealed large well defined exophytic heterogenous lesion arising from lateral wall of rectum with normal CEA levels. Biopsy revealed small round blue cell tumour which was positive for Pan CK and CD99. He was planned for 6 cycles of Neoadjuvant chemotherapy (NACT) with VAC IE followed by reassessment for local therapy followed by adjuvant chemotherapy with 8 cycles of VAC IE. He received 6 cycles of VAC IE and Definitive radiation therapy 45Gy in 25 fractions by 3DCRT. After completion of radiation therapy, he received 8 more cycles of VAC IE in TATA memorial hospital. We present this case report, due to the rarity of Extraosseous Ewing’s sarcoma (EES)of the rectum.
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