Background: This study aims to assess the quantity and quality of available literature on surgical treatment outcomes of spinal stenosis in adult and paediatric achondroplasia patients through a systematic review of literature and to investigate the suitability of conducting a meta-analysis on outcomes of surgical treatment. Methods: Online databases were searched according to PRISMA guidelines. No restrictions regarding study design, sample size, previous treatment, or publication date were implemented. The following terms: “Spinal stenosis”, “Spinal Decompression”, “Spinal fusion”, each term separately combined with the term “Achondroplasia” were used. Quality of the included studies were assessed used the Modified Coleman method. Results: Five adult and four paediatric single-sample non-comparative studies were identified for inclusion (176 adult and 102 paediatric patients). Meta-analyses assessed the proportion of patients achieving full resolution of symptoms to be 0.51 (95% CI 0.00 to 1.00); the proportion of patients achieving full or partial resolution of symptoms to be 0.90 (95% CI 0.84 to 0.97); the proportion of procedures requiring re-operation to be 0.42 (95% CI 0.34 to 0.50; and the proportion of procedures involving dural tears to be 0.20 (95% CI 0.02 to 0.39). Statistical heterogeneity was very high for full resolution of symptoms and requirement for dural repair; and very low for other outcomes. Conclusions: The available literature on this population and condition is sparse, highly heterogenous, and is generally of low quality limiting the value of meta-analysis. Overall, outcomes of surgical decompression of symptomatic spinal stenosis in achondroplasia patients show consistent degree of resolution of symptoms. Duration of symptoms prior to surgical treatment appears to play an important role in the overall outcome of treatment. Therefore, a delay in diagnosis and treatment can potentially be detrimental in achieving a better outcome.