Case: A 13-year-old girl presented with paresthesia of the fourth and fifth fingers and the dorsal ulnar surface of the left hand that had started 3 months prior. Physical examination showed loss of sensation at the ulnar side of the fourth and fifth fingers and a positive Froment sign. Electromyography showed a severe motor conduction block in the ulnar nerve at the elbow. Eighteen months later, the patient had similar symptoms in the right hand. The diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP) was considered and confirmed with genetic testing.
Conclusion: HNPP is a rare disease that should be considered not only in patients with multiple compressive neuropathies, but also in patients with any unexpected or unexplained neuropathy, even if it is isolated.