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Superficial siderosis (SS) is a rare, progressive neurological disorder with the most common symptoms being progressive sensorineural hearing loss, ataxia and myelopathy. We aim to report a rare case of the classical type of SS with early cognitive manifestation.An older lady presented with gradual onset hearing loss for 10 years, a decline in episodic memory, executive functions and visuospatial abilities, along with progressive worsening of ataxia and frequent falls for 2 years. After evaluation, a diagnosis of the classical variant of SS was considered. She was started on amantadine up to 200 mg/day, deferiprone up to 1000 mg/day, vitamin E supplementation and donepezil. There was a modest improvement in ataxia and frequency of falls.