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Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm of intermediate biological potential, most commonly affecting children and young adults, with the lung being one of the the most common sites. Its variable clinical, radiological and pathological features often mimic malignancy, making diagnosis challenging. We report a middle-aged man in his early 50s presenting with life-threatening haemoptysis and found to have a right main bronchus mass causing central airway obstruction. Emergency rigid bronchoscopy with cryo-debulking was performed for airway stabilisation and tissue diagnosis. Histopathology confirmed IMT. Immunohistochemistry was anaplastic lymphoma kinase negative, while molecular analysis demonstrated ROS proto-oncogene 1, receptor tyrosine kinase (ROS1) positivity, highlighting the molecular heterogeneity of IMT. Following multidisciplinary tumour board discussion, the patient was initiated on targeted therapy with crizotinib, with planned reassessment for surgical resection. This case emphasises the importance of early tissue diagnosis, molecular profiling and multidisciplinary decision-making in achieving personalised and potentially lung-sparing treatment.