One case of cervical myelopathy associated to ossification of transverse atlantal ligament (OTAL) and C1 posterior arch hypoplasia in a Caucasian adult female is reported. A 53-year-old female affected by cervical myelopathy was treated with C1 laminectomy and posterior arthrodesis. CT scan demonstrated that the distance between ossification of the ligament and anterior cortex of the posterior arch of atlas was 6,2mm leading to consistent space reduction for spinal cord at this level. Patient underwent spinal cord decompression and fixation with C1 poliaxial screws in lateral masses and two bilateral crossing C2 laminar screws with an improvement of neurological functions at 4-years follow-up. The association between OTAL and C1 hypoplasia was reported in very few cases. The treatment with C1 laminectomy without fusion is reported in medical literature with good clinical outcome. Our patient obtained a neurological improvement at midterm follow-up with spinal cord decompression and fusion.