A 59-year-old woman with nail patella syndrome (NPS) presented with progressive and severe right knee pain for the past 3 years. Imaging demonstrated laterally dislocated patella, with asymmetric femoral trochlea, and advanced lateral compartment arthritis. The patient underwent robotic-assisted lateral unicompartmental knee arthroplasty (RAUKA). The patient saw marked improvements in patient-reported outcome measurements from her initial visit to the last follow-up visit. While treatment of knee osteoarthritis in patients with NPS can be difficult, especially with attempts of patellar realignment, we present a case of a patient who underwent RAUKA, with sustained improvements in patient outcomes and range of motion, at the 2-year follow-up visit. This study is the first of its kind to demonstrate the use of a novel technology in a rare condition.