Introduction: Pancreatic carcinoma is extremely rare in pediatric and infrequently encountered in clinical practice. Their non-specific clinical presentation and overlapping imaging characteristics often make an accurate preoperative diagnosis difficult

Case presentation: 10 years old girl presented with one year history of abdominal discomfort, epigastric abdominal pain and abdominal lump noticed 2 months, there was no history of any other associated symptoms. On examination she had left supraumbilical transverse healed surgical scar and stoma. Pre operative abdominal CT scan suggests left renal tumour . She undergone laparotomy and found pancreatic tail tumour adherent to left kidney and spleen, histopathology confirmed acinar cell carcinoma of the pancreas. The girl starts on adjuvant chemotherapy modified Folfirinox protocol.

Conclusion: This rare tumor is being reported to highlight that such tumors arising from the tail of the pancreas and the adopted unconventional surgical approach and chemotherapy.Because of the small number of children diagnosed each year with pancreatic tumors, ongoing international cooperation is necessary for continued improvement in outcomes and for further advancement in our knowledge of the genetics and biology of these tumors.