Many patients with Pierre Robin sequence (PRS) have associated birth defects, most commonly in association with abnormalities in bone or cartilage formation. Depending on severity, treatment of PRS ranges from nonoperative management with prone positioning to surgical intervention such as distraction osteogenesis. Generally, if a surgical approach is needed, these patients undergo nasal endoscopy or direct laryngoscopy with their intubation, which puts the cervical spine in a position of extreme extension. The authors present a patient with syndromic PRS secondary to Sticklers syndrome, with a cervical abnormality diagnosed with three-dimensional computed tomography and further evaluated with dynamic lateral plain x-rays to assess cervical instability. The goal of this report is to highlight the need to include cervical spine evaluation in the preoperation workup of patients with PRS, especially those with suspected abnormalities in bone or collagen formation.