Moyamoya disease is characterized by narrowing of cerebral blood vessels and is progressive in nature. We present the case of a 21-year-old female patient who was a known case of Down's syndrome and presented with right-sided facial muscle weakness and was initially suspected of having cholesteatoma, but no evidence for the same was found after extensive radiological investigations. The suspicion of a central nervous system pathology was raised due to the clinical findings of dysphasia and supranuclear facial palsy. Magnetic-resonance angiography (MRA) was suggestive of findings of early Moyamoya disease. After a Neurologist consultation, the patient was started on conservative management with anti-platelet drugs and Statins and had symptomatic improvement. The patient was advised regular follow-up and had no fresh episodes of similar complaints.