Although classically associated with patients of East Asian origin, ossification of the posterior longitudinal ligament (OPLL) may cause myelopathy in patients of any ethnic origin. Degeneration of the PLL is followed by endochondral ossification, resulting in spinal cord compression. Specific genetic polymorphisms and medical comorbidities have been implicated in the development of OPLL. Patients should be evaluated with a full history and neurologic examination, along with cervical radiographs. Advanced imaging with CT and MRI allows three-dimensional evaluation of OPLL. Minimally symptomatic patients can be treated nonsurgically, but patients with myelopathy or severe stenosis are best treated with surgical decompression. OPLL can be treated via an anterior (ie, corpectomy and fusion) or posterior (ie, laminectomy and fusion or laminoplasty) approach, or both. The optimal approach is dictated by the classification and extent of OPLL, cervical spine sagittal alignment, severity of stenosis, and history of previous surgery. Anterior surgery is associated with superior outcomes when OPLL occupies >50% to 60% of the canal, despite increased technical difficulty and higher complication rates. Posterior surgery is technically easier and allows decompression of the entire cervical spine, but patients may experience late deterioration because of disease progression.