30 years ago Janetta proposed neurovascular compression as an etiology factor of trigeminal neuralgia. Neuroanatomical, neurophysiological studies and observations during neurosurgical operations seems to confirm Janetta's suggestions. The conception of neurovascular compression was accepted also as an etiology factor in hemifacial spasm. There are more diseases of unknown etiology described in literature in which neurovascular compression as an etiological factor is suspected. The results of initial treatment of these diseases performed by neurovascular decompression were presented. We are described the methods of contemporary diagnostics of neurovascular compression syndrome. The difficulties with correct diagnostics were underlined. MRI and neurophysiological examinations were pointed out as the most useful in an evaluation of neurovascular compression syndromes.