Background: Multiple Hereditary Exostoses with distal ulnar exostosis often results in forearm deformity and functional restrictions. Literature reported variable outcomes and frequent complications with an aggressive approach of ulna lengthening. The role of distal ulnar exostosis excision remained uncertain with contradictory effect in restoring growth. Our study aimed to report our outcome of simple excision and compare with the reported results in literature. Method: Retrospective review was carried out in patients with distal ulnar exostoses excision who were operated from 2003 to 2011. Patients with less than 3 years of follow-up, radial head dislocation or subluxation, or within 3 years of skeletal maturity were excluded. Clinical outcome including range of forearm rotation was documented. Radiological outcomes including radial articular angle, carpal slip, ulnar variance and radial bowing were measured. The results were analysed statistically and compared with literature. Results: Five cases were included in our study. All had excision of exostosis, and one case had additional ulnar lengthening. Mean age of operation was 10.4 years. Mean follow-up time was 72 months. Improvement was shown in the range of pronation, radial articular angle, carpal slip and ulnar variance, in the cases with simple excision alone. Improvements were mostly achieved at the first postoperative year. However, for the case with concomitant lengthening, ulna shortened relatively with growth, and complications were reported. This phenomenon is in keeping with the literature finding. Conclusion: Simple excision of distal ulnar exostosis improves forearm rotation and provides some restoration of growth in skeletally immature patients with Masada type I deformity.