Fractures of the axis body have been little reported and treatment strategies remain controversial and individualized. Not more than 10 cases of huge tear drop fracture of the axis (HTDFA) have been reported in previous studies and the treatment method varies from conservative treatment to an anterior, or posterior, approach surgery. Considering the sparse knowledge of HTDFA, we present a special case report to share our experience and to explore the safety and effectiveness of anterior reduction and fusion to treat HTDFA.A 24-year-old man was referred to our department; he presented with neck pain lasting for 12 h since being involved in a roll-over motor vehicle accident. His neck movement was limited but there was no neurological compromise. Physical examination of the patient showed myodynamia of four limbs Grade 5, Hoffmann sign (–), and Babinski sign (–). Three-dimensional reconstruction computed tomography (CT) confirmed a huge tear drop fracture of the anterior–inferior corner of the axis and discontinuity of the cortex of the axis. After discussion with the spinal surgeon team in the department and an effective conversation with the patient, surgery involving anterior reduction, discectomy, and three cortical iliac bone grafts with instrumentation after transnasal induction of general anesthesia was performed. The patient was instructed to wear a cervical collar until he returned to our department for a follow-up examination some 3 months after surgery. The 3-month postoperative x-ray and CT scan showed a good position of the implant and bony fusion at the C2/3 segment.Anterior reduction, discectomy, and three cortical iliac bone grafts with instrumentation to treat HTDFA are effective, safe, and simple. Of course, longer follow-up duration and more cases are warranted to verify this procedure. Anterior reduction, discectomy, and bone grafting with instrumentation are warranted for most HTDFA cases. However, if HTDFA incorporates other complex fractures, such as fracture of the posterior structure, an anterior and posterior union surgery is recommended.