Background: Inflammatory myofibroblastic tumour (IMT) is a very rare mesenchymal tumour with an unclear benign or malignant potential. Lung is the most common site of occurrence but less frequently it also occurs at various extra pulmonary sites. Common sites in abdomen include retroperitoneum and mesentery. Complete surgical excision is the standard of care and malignant variants need to be supported with adjuvant targeted therapy with ALK inhibitors. Case report: We present here a case of IMT of ileo-caecal junction presenting as frank acute intestinal obstruction in an 18 year old female which highlights the potential of this tumour for causing life threatening emergencies. Patient underwent laparotomy with right hemicolectomy. Histopathology was consistent with IMT of ileocaecal junction with no atypia. Patient was kept on regular follow up and presently she is asymptomatic. Conclusion: IMT is mainly a histopathological diagnosis and complete surgical excision with negative margins offers the best treatment. Owing to the risk of recurrence and malignant potential, regular follow up is mandatory and targeted therapy via ALK inhibitors is warranted in such situations