The authors present definition, classification and epidemiology of porphyria accompanied by neurological disorders. Diagnosis, treatment and prevention are discussed. The authors describe 3 young patients with newly diagnosed and laboratory-confirmed acute intermittent porphyria. In all cases, porphyria debuted with acute abdominal pain and was accompanied by various neurological disorders (polyneuropathy, epileptic syndrome, encephalopathy with mental disorders, vegetative state), proceeded under the mask of other diagnoses for a long time. In all patients, MRI of the brain revealed non-specific changes that were interpreted differently by radiologists. In two patients, dynamic monitoring of these changes in brain matter was presented for the first time. Considering own clinical data, the authors emphasize interdisciplinary nature of this issue, the role of awareness and alertness of physicians in early diagnosis of porphyria, importance of timely and modern therapeutic approaches for prevention of exacerbations of disease, severe disabling complications and mortality.