Tarlov cysts (perineurial cysts) are defined as cysts formed within the nerve root sheath at the dorsal root ganglion [
8], and most of them are clinically insignificant [
1,
7,
9]. The estimated incidence is approximately 5% (symptomatic cases are rare, constituting less than 1% of the total) [
10], and on MRI it has been estimated that Tarlov cysts are present in 4.6% to 9% of the population, with an estimated 10% becoming symptomatic at some point during life [
10]. Commonly Tarlov cysts are found in the lumbo-sacral region [
1,
4,
7,
9], with the S2/S3 nerve roots most commonly affected [
4,
7]. The exact etiology of perineurial cysts remains unclear. It was proposed that hemosiderin deposition caused by blockage of the venous drainage of the perineurium and epineurium after local trauma can lead to the development of these cysts [
11], or that congenital arachnoid proliferation along the exiting nerve roots can result in the formation of perineurial cysts [
12], and it has also been suggested that the ball-valve mechanism is responsible for the entry of cerebrospinal fluid into the cyst during systolic pulsations but that the cerebrospinal fluid is unable to exit through the same portal during diastole [
7,
9,
11]. A histological characteristic of the Tarlov cyst is the presence of nerve fibers in the cyst wall [
1,
7,
8,
9]. Symptomatic Tarlov cysts are rare and clinical symptoms depend on the location of the cyst; symptoms range from backache, perineal pain or sciatica to overt cauda equina syndrome [
4,
9]. The symptoms are mostly exacerbated by maneuvers that elevate the intraspinal cerebrospinal fluid pressure, including coughing, walking, change of posture, and the Valsalva maneuver [
13]. In the present case, the patient had features of compressive cervical myelopathy because of the location of the cyst. MRI is an effective way to investigate these lesions as it will provide better details such as showing the extent of the lesion and its relationship to surrounding structures [
3,
6,
10]. For symptomatic cases microsurgical excision of the cysts is curative and has a good outcome [
3,
4,
7]. In the present case, because of the rarity of the lesion, we did not suspect a Tarlov cyst at first; however, the complete microsurgical excision resulted in a good outcome.