The authors describe a case of hereditary neuropathy with liability to pressure palsies (entrapment), and compare it to reports from literature. The main characteristics are: autosomal dominant inheritance, recurrent mononeuropathies (ulnar, median, peroneal, brachial plexus), and specific features at nerve biopsy. The sensory nerve reveals predominantly demyelinating alterations, having the remaining myelin fibres focal thickenings, the so called tomaculous, and shows numerous subperineural structures named Renaut Bodies. The EMG findings show a slowing of the nerve conduction velocities and an increase of distal latencies.