Purpose <p>Individuals with achondroplasia are prone to lumbar stenosis with a common need for spinal decompression surgery. However, given anatomical and medical factors, this population is at increased risk for surgical and anaesthetic complications, thus posing a unique challenge to the clinicians. The use of an awake minimally invasive endoscopic technique under monitored anaesthesia care may prove beneficial to mitigate such risks. We present a case of a 52 year old female with achondroplasia suffering from left radicular leg pain. A Magnetic Resonance Imaging scan showed severe lumbar spinal stenosis and lateral recess stenosis at L4/5 and L5/S1, with compression of the descending L5 and S1 nerve roots respectively. There was also moderate foraminal stenosis at both levels with compression of exiting L4 and L5 nerve roots respectively.</p> Case presentation <p>The patient underwent awake endoscopic decompression utilising a uniportal interlaminar endoscopic system under local anaesthesia with sedation.</p> Results <p>The patient recovered well post-operatively, with immediate resolution of her symptoms. She was ambulating up to 100&#xa0;m on postoperative day one.</p> Conclusion <p>The awake endoscopic procedure described is an option that may be well suited for patients with achondroplasia presenting with lumbar stenosis.</p>