The idiopathic generalized epilepsies (IGE) occur with a high aggregation within families. Juvenile myoclonic epilepsy (JME) is recognized as a commonly occurring form of idiopathic generalized epilepsy. A possible association between JME and HLA antigens was investigated by serological typing of human leukocyte antigens (HLA) class I antigens and by DNA oligotyping of class II antigens. Twenty-four patients and 129 controls, all Caucasians of Scandinavian descent, were tested. Uncorrected there was a significant positive association (Relative risk (RR) = 8.07) to B17 and a significant negative association (RR = 0.13) to B8 as well as DRB1*3. The negative association to DQ alleles DQA1*0501 and DQB1*0201, which are in strong linkage disequilibrium with the alleles B8 and DRB1*3, was weaker and not significant, thus giving no clue as to a primary HLA-DQ association of JME.