Purpose To systematically evaluate any consensus for the etiology, definition, presentation and outcomes of developmental lumbar spinal stenosis (DLSS). Methods A comprehensive literature search was undertaken by 2 independent reviewers with PubMed, Ovid, and Web of Science to identify all published knowledge on DLSS. Search terms included “developmental spinal stenosis” or “congenital spinal stenosis” and “lumbar”. The inclusion criteria were English clinical studies with sample size larger than 8, articles examining the etiology, diagnostic criteria, surgical outcomes of DLSS, and its association with other spinal pathologies. Articles that did not specify a developmental component were excluded. The GRADE approach was used to assess their quality of evidence. Results The initial database review found 404 articles. Twenty articles with moderate to very low quality met the inclusion criteria for analysis. The bony canal diameter was significantly shorter in patients with DLSS than normal subjects. In addition, the risk of re-operation on adjacent levels (21.7%) was high which could be explained by multi-level stenosis. However, there was a lack of consensus on the methodology of diagnosing DLSS and on its specific surgical techniques. Conclusion Multi-level stenosis and re-operation at adjacent levels are especially common with DLSS. Identification of these individuals provides better prognostication after surgery. However, current literature provides few consensus on its definition and the required surgical approach. Besides, there are limited reports of its etiology and association with other spinal pathologies. Due to these limitations, standardizing the definition of DLSS and investigating its etiology and expected clinical course are necessary.