Background: Most of the first symptoms of avian influenza are respiratory symptoms, and cases with occipital neuralgia as the first manifestation are rarely reported.

Case summary: A middle-aged patient complaining of paroxysmal pain behind the ear was admitted to our hospital. The patient´s condition changed rapidly, and high fever, unexpected respiratory failure, and multiple organ failure developed rapidly. The patient was diagnosed with H7N9 avian influenza based on etiology.

Conclusion: We believe that the etiology of occipital neuralgia is complex and could be the earliest manifestation of severe diseases. The possibility of an infectious disease should be considered when occipital neuralgia is accompanied by fever. Avian influenza is one of these causative agents.