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Background and objective: Globally, diastematomyelia accounts for 5% of all congenital spinal cord defects. Clinically, symptoms of diastematomyelia are non-specific, can be progressive, and can become symptomatic at any age. This study was carried out to describe neurological, radiological and surgical findings of diastematomyelia cases reported in Erbil city of Iraq. Methods: A retrospective review of diastematomyelia was carried out in three neurosurgical centers in Erbil city of Kurdistan region of Iraq between 1st January 2003 and 1st January 2013. Diagnosis of this anomaly was based on CT and MRI with surgical dissection in one case. In patients with contraindication for MRI and CT scan, the diagnosis was based on lumbar myelography. Surgical interventions included surgical decompression and laminectomy with timely follow-up at every six months to assess the outcomes after the surgical intervention. Results: A total of 27 cases were included in this study with a mean age of 13 years (ranging from 1-19 years) and a female to male ratio of 2.9:1. Spinal deformities (66.7 %) were the main complaints for patients to seek medical advice. Clinically, 89 % of the patients had a huge spinal disfigurement, and 74% had a mid-line thoracic or lumbar cutaneous variation from the norm. Neurologically, 59 % of the patients had a least neurological disability. Radiologically, 96 % showed inter-pedicular separation and spina bifida, 59% scoliosis, and 55.6% boney spicule. Intra-operatively, around 63% of the cases had boney septum separating the dissected hemi-cords; 70 % of the septa located in the lumbar region. Post-operatively, none of the patients experienced decay in their neurologic status after surgery. Over the long term, two patients were slightly improved, and another two had an increased neurological deficit, one patient had better reflexes, but increasing deformity of the foot and 14 patients were unchanged. None of the patients had contamination, pseudoarthrosis, or loss of remedy amid the subsequent visit. Conclusion: Despite the slight postoperative improvement, all patients with the established preoperative deficit still had residual neurological postoperative deficits and only a low proportion of them slightly improved.