Sympathetic ophthalmia (SO) is very rare but it remains one of the most intractable eye diseases. In clinical manifestations and histopathologic features SO is known to resemble closely Vogt‐Koyanagi‐Harada's disease (VKH disease). We had reported that VKH disease was significantly associated with HLA‐DRB1*04 and ‐DQB1*04 in Japanese patients. In this study, to investigate an HLA association with SO we performed HLA serological and PCR‐based DNA typing in 16 patients and 50 healthy controls. Our study revealed that HLA‐DRB1*04 (0405; Pc<5×10A DQA1*03 (Pc<5×10^‐3), and DQB1*04 (0401; Pc<5times10^‐4) were significantly associated with SO as compared to the healthy controls but there was no significant difference in the frequencies of any DPB1 alleles between the patients and healthy controls. It can be postulated that not only the clinical manifestations but also the genetic predisposition of SO are very similar to those of VKH disease.