Patients with tethered cord syndrome, especially children, should be alerted to one clinical manifestation of the disease: hairy, abnormally pigmented skin in the lumbosacral region. Tethered cord syndrome (TCS) is a syndrome in which the spinal cord or conus is stretched due to various congenital and acquired causes, resulting in a series of neurological dysfunctions and deformities. TCS is also known as hypospadias because the spinal cord is mostly stretched in the lumbosacral medulla, causing the cone to be abnormally low. The diagnosis of spinal cord embolism syndrome is not difficult based on the typical medical history, clinical manifestations and ancillary examinations. Since the disease is often asymptomatic or insidiously developed in the early stage, a few patients with acute onset cannot improve neurological dysfunction even after treatment. Therefore, it is important to raise awareness of the disease, achieve early diagnosis and timely treatment. Diagnosis of lumbosacral skin hirsutism and abnormal pigmentation: ① pain is widespread and cannot be explained by damage to a single nerve; ② adults have obvious triggers before the onset of symptoms; ③ bladder and rectal dysfunction and frequent urinary tract infections; ④ progressive worsening of sensorimotor disorders; ⑤ different congenital malformations or a history of lumbosacral surgery; ⑥ MRI and/or CT spinal canal imaging reveals spinal cone abnormal position and/or thickening of the end filaments.