The development of an hourglass-like constriction in the nerve is rare, and its origin is unknown. Its clinical manifestations are not well documented, and the treatment protocol has not been established.To identify the cause, presentation, and possible treatment for patients with nerve palsies secondary to an hourglass-like constriction in the affected nerves.Patients presenting with peripheral nerve palsy caused by an hourglass-like constriction of nerves were retrospectively investigated in 2 hand centers. The patients' presentation and neurological findings were reviewed, and the immunohistochemistry of excised specimens was studied.Forty-two patients who presented with 47 nerve palsies were examined. Forty-one patients experienced a sudden onset of pain in the upper limb, followed by flaccid paralysis in the affected muscles. Ten patients had multiple nerve involvement. Surgical exploration found 1 or more hourglass-like constrictions in the nerve. The treatments included internal neurolysis, neurorrhaphy, and nerve grafting. Thirty-one of 42 patients (36 nerves) were followed up for a mean of 48 months (range, 8-157 months). Fifteen of 16 nerves treated by neurolysis, 10 of 13 nerves treated by neurorrhaphy, and 4 of 7 nerves treated by nerve grafting had good recovery. CD8-positive T-lymphocyte infiltration was observed in all the excised specimens.The clinical presentation of patients with hourglass-like constrictions in their nerves is similar to that of patients with neuralgic amyotrophy. Histochemical analysis suggests that the pathogenesis may be immunological in origin. The role of surgery in this condition is uncertain.