Background: Open fractures with bone defects of the distal phalanx of the hallux are both rare and complicated, and these conditions pose great clinical challenge to orthopedists as improper handling can lead to distal tissue necrosis, osteomyelitis, malunion, non-union and even amputation. Due to the lack of soft tissue and bone and the blocking effect caused by un-disconnected soft tissue, it is relatively difficult to achieve effective reduction and fixation of the fracture by traditional methods. Methods: we present a patient, diagnosed with open fracture combined with bone defects of the distal segment of the great toe, who was treated using a self-made adjustable external fixation device by complete debridement, effective closed reduction and continuous adjustment. Complications were registered and clinical outcome was measured at the follow-up periods. Results: The device was simple to make with a low cost and practicality, and this technique resulted in effective maintenance of reduction and progressive compression in the fracture ends. The patient finally obtained optimal fracture healing and satisfactory function of the injured toe at last follow-up. Conclusions: The selfmade adjustable external fixation device is reliable in maintaining reduction and fixation, which provides an alternative for the management of open fracture associated with bone absence of the distal phalanx of the hallux.