Purpose <p>Pediatric colorectal malignancies are rare, with lymphomas- predominantly non- Hodgkin’s lymphoma (NHL) representing the most common type, accounting to 0.5-2% of large bowel malignancies. Colorectal NHL can be primary or secondary. This study analyzes the rare manifestations of pediatric colorectal lymphoma and examines their diagnostic challenges and surgical implications.</p> Method <p>A Pubmed search was done with search words “lymphoma of colon pediatric” with filter of last 5 years on 12 Feb 2026. The search comprised of 47 articles, among which 10 relevant articles were selected. After going through the articles, one article was excluded. 9 articles were finally included in the study.</p> Results <p>Colorectal lymphoma can be a primary malignancy or it can arise in the background of inflammatory bowel disease. It can also be a secondary extension from bulky intra- abdominal lymphoma. Clinical manifestations can present either as flare up of inflammatory bowel disease or colorectal carcinoma or bowel obstruction or acute abdomen. This can lead to potential diagnostic delay and inappropriate escalation of medical therapy, unless early biopsy is performed. The routine use of broad spectrum anti-infectives in lymphoma decreased the gastrointestinal integrity and increased opportunistic infections in the gut, complicating the clinical assessment. Imaging aids in differentiation from epithelial malignancies. It also guides in staging, treatment and operative planning. Rare presentations of colonic lymphoma were its appearance in a child (below five years) on medical management for ulcerative colitis, and in another patient on Vedolizumab for Crohn’s disease. Another rare presentation recorded occurrence of primary rectal lymphoma in an eleven year old child. Early presentation (below 10 years) with synchronous colorectal carcinoma and family history of malignancy, suggested an underlying genetic cancer predisposition syndrome. Surgery plays a crucial role in managing complications like perforation, obstruction and intussusception when lymphoma is the lead point. Laparoscopy assisted en-bloc resection is feasible in selected cases.</p> Conclusion <p>Pediatric colorectal lymphomas represent a clinically significant and occasionally misleading entity. While systemic chemotherapy remains the cornerstone of treatment, surgical intervention has a defined role in tissue diagnosis and management of complications. Early recognition and coordinated multidisciplinary care are essential to optimize outcomes.</p>