Aim to investigate for the first time whether the regenerative approach can be used to rehabilitate a case with regional odontodysplasia (ROD). ROD is a rare, localized developmental anomaly of the dental tissues. Moreover, we review the various treatment protocols for ROD and compare them to the suggested regenerative protocol. Case Report A 22‐year‐old female patient diagnosed with ROD in the upper left quadrant was presented to our clinic. Initially, the affected teeth were extracted and three implants were inserted. A combination of autologous bone marrow mononuclear cells (BMMNCs) seeded on a collagen sponge, nanohydroxyapatite, and autologous platelet‐rich fibrin (PRF) was used to enhance bone regeneration in the defective area and around the inserted implants. After 9 months, bone regeneration and successful osteointegration around the inserted implants were achieved, permitting the insertion of a fourth implant. After an additional six months, a final fixed restoration was constructed. Conclusion The suggested regenerative approach provides a better treatment option for ROD patients to regenerate the lost bone, rehabilitate aesthetics, and restore normal function.