PURPOSE: "Benign Infantile Convulsion with Diarrhea" was first described by Morooka in 1982. Even without fever, dehydration, electrolyte imbalance and intracranial infection, seizures can occur several times in a day after the onset of diarrhea. Except for the seizures during the patient's acute illness, neurological outcome is excellent and no patient suffers epilepsy. Because seizures can occur repeatedly without accompanying fever, and with only mild diarrhea, these patients can be misdiagnosed with infantile epilepsy and treated with unnecessary anticonvulsants. METHODS: This study was carried out with 61 patients with benign infantile convulsion with diarrhea who visited at Inje University Sang-gye Paik Hospital, from September 1989 to June 1997. RESULTS: There were 61 patients with benign infantile convulsion with diarrhea, 32 boys and 29 girls. Sixty out of 61 patients had generalized tonic-clonic seizures, and one patient showed partial seizure with secondary generalization. Sixteen(45.7%) out of 35 patients showed positive rotazyme tests. Cerebrospinal fluid examination showed normal in 14 patients, EEG's were normal in 38(84.4%) out of 45 patients, brain CT's were negative in 24 out of 25 patients, and brain MRI's were negative in 4 patients. We could follow up 38 patients, and 3 patients had recurring seizures due to benign infantile convulsion with diarrhea. Psychomotor developments were normal in all the follow-up patients. CONCLUSION: Benign infantile convulsion with diarrhea is a benign seizure disorder in infancy without relation to later neurological disability or epilepsy.