Neurodegeneration with brain iron accumulation is a rare group of neurodegenerative diseases that present extrapyramidal symptoms, mood disorders, and cognitive impairment. A prevalence of 1 to 2 cases per million inhabitants is estimated. In Latin America, there is no estimated prevalence, although there are some reported cases with typical presentation. The first case report of this disease in Ecuador is presented, in an atypical presentation, under the ruling out of differentials and compliance with diagnostic criteria.

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