Purpose: Tuberous breast is a complex congenital breast anomaly which can be challenging to correct surgically. Using a new classification system, we conducted a systematic review with pooled analysis of data with an aim to determine the effectiveness and complications related to operative management of the deformity. Methodology: Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were used in performing this systematic review. A simplified classification system for tuberous breast deformity was developed to accurately compare data and guide analysis. Results: The review identified 38 studies reporting a total of 897 patients undergoing tuberous breast surgery. The mean age of patients was 24. Mean follow up was 39 months. A combination of tissue rearrangement and implant augmentation was the most used technique followed by fat transfer alone. Breast implants were used in 83% of patients. The mean implanted volume per breast was 263cc. Fat grafting was performed in 13% of patients. The overall complication rate of 20%. Subjective assessment of patient satisfaction was 99% and the mean score using BREAST-Q for satisfaction with clinical outcome was 86.7. Future studies should focus on robust study designs including randomised and cohort studies, use of patient-reported outcome measures and long term follow up. Conclusion: This new classification allowed accurate collation of data from existing literature in order to facilitate the systematic review. The surgical techniques to correct tuberous breast deformity are safe, effective and have a high satisfaction rate. Fat transfer has the capacity to provide promising results in tuberous breast deformity.