To describe the case and management of a patient with marchiafava bignami disease (MBD) with frontal cortical lesions, no specific symptoms at first referral to the emergency room, and late onset of atypical psychiatric symptoms.

We report the case of a 44-year-old patient with a history of chronic alcohol abuse, eventually diagnosed with MBD.

Magnetic resonance showed lesions in the splenium and in the body of corpus callosum and bilateral lesions of the frontal cortex. The patient showed late-onset atypical psychiatric symptoms, which were drug resistant.

The case we describe seems to support the existing few ones describing cortical involvement in MBD, which suggest that this is associated with a poorer prognosis. Psychiatric symptoms may be challenging to treat because of drug resistance. The involvement of psychiatrists together with neurologists and radiologists, with a consultation–liaison approach has proved important for the achievement of diagnosis and of the most appropriate management and treatment for this patient.

The authors have not supplied their declaration of competing interest.