We report a case of complete Heerfordt syndrome accompanied by the involvement 
of small fiber neuropathy (SFN) manifesting as refracory facial pain. A 
30-year-old man presented with pyrexia, a 2-week history of facial burning pain, 
and difficulty of mastication. After admission to our hospital, neurological 
examinations showed bilateral facial pain, trigeminal motor palsy, left facial 
nerve palsy, bilateral sensory neural deafness, uveitis and swelling of the 
parotid gland. Other examinations revealed bilateral hilar lymphadenopathy, high 
serum titer of angiotensin coenzyme, and no response in a tuberculin-tested, 
non-caseating epithelioid granuloma from lip biopsy, leading to the diagnosis of 
complete Heerfordt syndrome. Mandibular skin biopsy with immunostaining for PGP 
9.5 showed SFN. High-dose corticosteroids proved somewhat effective against SFN 
as facial pain, but reducing the corticosteroid dose proved difficult, as 
symptoms were refractory to other immunosuppressants and pain-control drugs such 
as anti-epileptics and anti-depressants. The patient died of acute pancreatitis 
3 years after disease onset. Autopsy showed no granuloma in hilar lymph node, 
trigeminal nerve, cranial base, nerve root, and muscle. SFN in this case 
probably represent a cause of refractory facial pain.