Chorea minor is the most curious manifestation of rheumatic fever, first described by Sydenham in 1686. Subsequent evidence showed that chorea could be a late manifestation of rheumatic fever, often occurring several months after a streptococcal infection in contrast to other major manifestations. During the ten-year period between 1984 and 1993, 11 children with rheumatic fever were seen at our hospital, two cases of these being accompanied with chorea minor. Case 1, a male aged 12, presented with involuntary movements. He was diagnosed as having rheumatic fever because of chorea and systolic ejection murmur at the apex of the heart. Plain cranial CT was normal. However, positron emission computed tomography revealed an increased 11C-glucose uptake in the caudate nucleus as compared with the cerebral cortex. Case 2, a female aged 14, presented with involuntary movements. Plain cranial CT was normal, but single photon emission CT showed a difference between the right and left brain. These two patients were given penicillin G (PCG), predonisolone (PSL) and haloperidol. Haloperidol was administered, because PCG and PSL had no effect to improve the clinical manifestations. After administer decreased to a half with a clinical improvement, but the level of GABA did not change. Haloperidol seemed to be an effective and useful agent for motor manifestations of the disease.