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Background: Langerhans cell histiocytosis (LCH) has a variable presentation. Gastrointestinal involvement is rare and portends a poor prognosis in LCH. Case report: Herein, we describe the presentation, progression, management, and outcome of an 11-month-old female infant with LCH. The patient presented with severe acute malnutrition, hepatosplenomegaly, and gastrointestinal involvement in the form of persistent vomiting. She was managed with chemotherapy initially and subsequently developed complete duodenal narrowing. She underwent Kimura's duodenojejunostomy and improved. Hence, chemotherapy was restarted. Two months later, she developed features of intestinal obstruction with intraoperative findings of ileal gangrene requiring adhesiolysis, ileal resection, and ileostomy. Subsequently, she succumbed to sepsis. Conclusion: This case report aims to highlight the atypical gastrointestinal manifestations of LCH and the challenges faced in managing such a case.