This study aimed to evaluate the clinical feasibility and effectiveness of a monoaxial screw-rod system and anterior screw fixation for C1 and type II odontoid fractures. We conducted a retrospective review of 2 consecutive patients with acute C1 and Anderson-D'Alonzo type II odontoid fractures. Both patients underwent treatment using a posterior monoaxial screw-rod system and anterior screw fixation. We reviewed their clinical records, including the visual analog pain scale and Neck Disability Index scores, as well as pre- and postoperative radiographs. Additionally, pre- and postoperative computed tomography images were used to classify the fracture types and assess the C1 to C2 reduction, rotation, and instability. Both patients presented with type II C1 and type II B odontoid fractures, combined with Dickman type II transverse atlantal ligament injuries. All surgical procedures were successfully performed without complications such as vertebral artery injury, neurological deficit, esophageal injury, or wound infection. Both patients achieved almost complete bone healing of the fractures, and C1 to C2 rotation was well preserved (32° and 49°) without atlantoaxial instability after follow-ups of 21 and 25 months, respectively. A monoaxial screw-rod system and anterior screw fixation could be promising surgical strategies for C1 fractures combined with type II odontoid fractures, even in cases involving transverse atlantal ligament injuries. The preservation of C1 to C2 rotation without atlantoaxial instability was observed after fixation. However, extensive case-finding and long-term follow-up are needed to understand the effectiveness of this treatment. In order to preserve the C1-C2 rotation, a monoaxial screw-rod system and anterior screw fixation may be more suitable for patients with C1 fractures combined with type II odontoid fractures.