We present the case of a 24-year-old man diagnosed with a neuroacanthocytosis, obsessive-compulsive disorder, mild-moderate severity depressive disorder, and obsessive personality disorder. He received multiple psychotropic drugs, including neuroleptics, antidepressants, and benzodiazepines with small therapeutic response.Severe and progressive clinical impairment appeared including cognitive deterioration, chorea, tics, lingual dystonic protrusion, Parkinsonism, automutilations, and other behavioral disorders consistent with neuroacanthosytosis. Pharmacological trials were not efficacious, and the patient had medication side effects, so pharmacotherapy was reduced to minimal doses and we began electroconvulsive therapy.When the sessions were finished, the patient's speech was more coherent, although the neuroacanthosytosis was unstoppable in its progression. Thereafter, the patient received continuation electroconvulsive therapy fortnightly for 4 months until he died due the neurodegenerative disorder.