Abstract Background Meningoencephalitis by Cryptococcus gattii (C. gattii) can be responsible for unspecific neurological clinical features, and its lesions in the brain can be diffuse with a preference for basal ganglia. Case presentation Here we describe the case of a 72- year-old woman who developed dementia and akinetic rigid syndrome with bilateral hand myoclonus in six-month period. Brain magnetic resonance imaging revealed striking leptomeningeal compromise and lesions in the midbrain. Film array and culture for C. gattii in cerebrospinal fluid were positive and she was diagnosed with meningoencephalitis by C. gattii. Antifungal management with amphotericin B plus flucytosine was started, and induction therapy was proposed for 4 to 6 weeks. Conclusions Our case showed that meningoencephalitis by C. gattii associated with rapidly progressive dementia, gait disturbance and a pseudo-parinaud syndrome could be an atypical clinical presentation of meningoencephalitis by cryptococcus.