INTRODUCTION AND IMPORTANCE: Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic condition that primarily affects children and young adults. This case report highlights the challenges in diagnosing and treating pediatric IMT, emphasizing the importance of tailored interventions. CASE PRESENTATION: An 8-year-old boy presented with respiratory symptoms and was diagnosed with an IMT located in the left main bronchus. Imaging studies revealed a soft tissue mass and lymph node enlargement. Bronchoscopy and biopsy confirmed the diagnosis. The patient underwent bronchoscopic debulking procedures followed by lobectomy and bronchoplasty due to persistent disease. CLINICAL DISCUSSION: IMTs pose diagnostic challenges due to their varied clinical presentation and similarities with other neoplasms. A multidisciplinary approach involving pathologists, radiologists, and surgeons is crucial for accurate diagnosis and optimal treatment planning. The pathogenesis of IMTs is not fully understood, but theories suggest an inflammatory response or involvement of the ALK gene. IMTs can affect various organs, each with distinct symptoms. Imaging modalities lack specificity, emphasizing the importance of histopathological examination. CONCLUSION: IMTs require individualized treatment approaches based on the location and extent of the tumor. Long-term follow-up is essential for monitoring recurrence and metastasis. Further research is needed to enhance our understanding of IMT biology and develop targeted therapies to improve patient outcomes. This case report underscores the importance of tailored interventions in pediatric IMT cases and highlights the challenges in diagnosis and treatment.