Aim: Our aim is to report the successful treatment of an intraosseous haemangioma of tibia with an atypical presentation through a multidisciplinary approach of preoperative embolisation, a subtotal resection of the tibia and subsequent reconstruction with the Ilizarov medial fibular translation technique. Background: En bloc excision is the treatment of choice for large tumours of the tibia. However, there is no single recommended method for the reconstruction of the resulting bony defect. Case: A 22-year-old female presented with a massive intraosseous haemangioma of the entire tibia. Sequential, multimodal treatment consisted of (1) preembolisation, (2) en bloc resection and (3) reconstruction of the extensive skeletal defect via the Ilizarov method of fibular medialisation. Radiologic union occurred at 6 months and graft hypertrophy at 22 months. At 45 months, the patient was fully weight-bearing without need for an assistive device. Conclusion: Resection and reconstruction of a large intraosseous haemangioma of the tibia can be treated successfully using a well-planned sequential management of embolisation, resection and Ilizarov fibular grafting. Significance: This report highlights the successful management of an unusually extensive and difficult tumour through appropriate and meticulous perioperative multidisciplinary planning, execution and follow-up.