Spinal dysraphism is characterized by a lack of fusion of the vertebral arches that occurs in the absence of spinous processes with variable amounts of lamina. Here, we retrospectively present the importance of cutaneous lesions and their correlation with clinical presentation, radiological examination and urodynamic assessment. We retrospectively reviewed 612 (6.12%) cases with skin lesions from 10,000 consecutive live-born children seen at two institutions between January 1998 and March 2005. We divided all children into a control group and three groups based on clinical assessment, radiological examination and urodynamic evaluation results. Neurological deficits were identified in 113 (18.46%) children, while spinal dysraphism disorders, tethered cord syndrome and associated orthopedic malformations were found in 171 (27.94%), 119 (19.45%) and 28 (4.57%) patients, respectively. The incidence of tethered cord with lumbosacral dimple lesions over the sacrum (32/119, 26.65% of patients) was 3.5-fold higher than that of lesions found over the coccyx (9/119, 7.5% of patients). Uroneurological symptoms were found in 207/612 (33.82%) children. Urodynamic assessment revealed decreased bladder capacity in 10% of patients, detrusor hyperflexia during filling in 47% and a low-compliance detrusor in 71%. Discordance between ultra- sonography and MRI was found in 16.58% of patients. Spinal cord untethering was performed for 109 patients. Nearly all children with resolution were at the end of their follow-up period (24 months). Retethering occurred in 21 (19.26%) patients, and a second untethering surgery was performed in 12 patients. When spina bifida was associated with lumbar skin lesions, there may have been an increased incidence of tethered cord and other spinal cord disorders. MRI scans are more reliable and give an exact diagnosis of tethered cord. Neurological and uroneurological instability are ultimately a clinical diagnosis, and there is controversy about their indications for surgery. However, the correlation between urodynamic assessment and cutaneous lesions with a tethered cord found by MRI examination allow for an early diagnosis and the possibility of prompt treatment.