Various case reports of patients with Dandy-Walker variant syndrome mimicking schizophrenia have been published in the last 20 years, suggesting that this neurodevelopment disorder should be part of the differential diagnosis for every patient with a first episode of psychosis. In this report, it is presented that a patient who was diagnosed and treated as a paranoid schizophrenic for 20 years and had four bone fractures secondary to ataxic gait impairment, before performing a neuroimaging examination that revealed Dandy-Walker variant syndrome.