A 6-year-old boy developed rhabdomyolysis following hyperthermia and status epilepticus with a diagnosis of severe myoclonic epilepsy of infancy. At 2 and 3 years of age, he had similar episodes. Each time he recovered completely in 3-4 weeks with conservative management, in spite of renal insufficiency and marked liver dysfunction. Several cases of recurrent myoglobinuria after intense exercise of generalized tonic-clonic convulsions were reported to have genetic errors of carbohydrate or lipid metabolism of muscle. In our patient, however, the activity of these enzymes was found to be normal. This indicates that status epilepticus may cause recurrent rhabdomyolysis in subjects with normal glycolytic and lipolytic enzyme activity.