Dental problems in a patient with osteopetrosis pose a challenge from dental treatment because of associated co-morbidities and treatment regimes. Objective: This case gave an insight into the treatment and management of a patient with mandibular and palatal exostosis with osteopetrosis. Methods: The following information was obtained, a medical and surgical history, history of the main complaint, habits of the patient, radiographs, blood parameters, liver functioning tests, partial thromboplastin time, International Normalized Ratio, urea and electrolytes. Primary, secondary, extraoral and intraoral examinations were performed. A treatment plan was drawn up and the procedure was undertaken. Prophylactic antibiotics were administered both preoperatively and postoperatively to minimize the risk of osteomyelitis or any other infection. Results: Examination showed bony protuberances that were present at birth. The main complaint was interference of exostosis for denture fabrication and generalized bone pain for approximately twenty years. As a result the patient was on chronic pethidine and decadron for pain. The patient had osteopetrosis with lingual mandibular tori and lobulated palatal tori. The treatment plan involved surgical removal of tori and alveolectomy under general anesthetic. Healing was uneventful and the patient was referred to the Department of Prosthetic Dentistry for assessment and denture fabrication. Conclusion: The patient was diagnosed with an intermediate variety of osteopetrosis. The two conditions osteopetrosis and exostosis present in this patient were apparently not linked. With adherence to prophylactic antibiotics and surgery undertaken in two stages at different appointments, in order to avoid compromising the blood supply to the palatal mucosa, the prognosis of the surgery was positive.