Spinal arachnoid cysts are uncommon benign lesions that occur in the spinal axis, and can cause spinal cord or nerve roots compression and may become clinically relevant.The authors report a 49-year-old woman who presented progressive onset of cervical pain with distal paraesthesia of the upper limbs associated to an intramedullary arachnoid cyst in the mid-cervical spine diagnosed by MRI.From the literature review, only six cases in patients over 18-year-old were found, all with thoracic location and one with cervical extension, and all were submitted to decompressive surgery.The authors described the first intramedullary arachnoid cyst with a wait and scan approach.