Inflammatory myofibroblastic tumours (IMT) are rare, challenging lesions with respect to differential diagnosis, biological behaviour and treatment. We reviewed the literature and report a unique case of a large (9 × 8 × 8 cm) IMT in the nasal region of a 6-year-old girl responsible for important facial deformation. Following surgical resection, without any craniofacial reconstruction, the dysmorphism regressed spontaneously with a good result at 2 years of follow-up. To date, this is the largest IMT reported. Surprisingly, after simple excision of the tumour the craniofacial bones and soft tissues regained spontaneously their normal anatomical position only after a few months. The bone plasticity was likely due to the young age of patient and the preservation of periosteum and muscles during the surgical excision. We conclude that a conservative approach may be considered as alternative to reconstructive surgery in particular cases in young subjects.