Introduction: Moyamoya disease (MMD) is a rare neurovascular disorder that is characterized by chronic progressive stenosis at the apices and bilateral occlusion of the intracranial internal carotid arteries. The occlusion is gradual in manner; multiple anastomoses are formed between the internal and external carotid arteries. The incidence of MMD has increased in Asian countries with a decline in Europe and United States. Case description: The case report describes the specific clinical features and dental treatment in a 12-year-old male presenting with MMD with right-side hemiplegia. The patient presented with broken tooth in the upper front tooth region due to trauma. The dental treatment plan focused on providing restorative as well as preventive care to the patient. Clinical significance: The patients with MMD have an increased risk of stroke development, which may be aggravated by pain or anxiety. It is necessary to avoid cerebral ischemic attacks caused by fluctuating BP and respiration. It becomes imperative to maintain normocapnia, normotension, and pain control during the procedure in order to prevent postoperative ischemic complications. Dental professionals must attain a complete knowledge of this disease and perform the dental treatment in pain and stress-free manner.