Three children reported here and an additional five patients from the literature with angiomas of the pons had a total of 19 clinical episodes of neurologic “illness.” Thirteen of these episodes were ictal and six were progressive in onset. The progressive onset was identical with that occurring in patients with tumors of the brain stem. Laboratory and radiographic studies were not uniformly helpful in differentiating this disorder from the more common brain-stem glioma. The survival of these patients varies from several hours to 15 years.