We studied the neuropathology of 7 infants who had primary respiratory problems unrelated to increased intracranial pressure. These infants ranged in age from newborn to 2 years. Five were male. In 2 of them the main neuropathological findings were in the brainstem with prominent neuroglial heterotopia in the subarachnoid space, and aplasia of the VI and VII cranial nerves. Two infants had abnormalities of the X and XII nerves together with neuronal heterotopia and migration failure of the inferior olivary nuclei. In 1 infant diagnosed with Ondine's curse, examination showed diffuse neuronal loss and gliosis in the medullary tegmentum. One infant had a unilateral infarction in the medulla and another showed extensive gliosis in the brainstem tegmentum along with a large infarction in the region of the anterior cerebral artery. These infants exhibited a spectrum of abnormalities including neuronal dysplasia, gliosis and hypoxic-ischemic changes. In the differential diagnosis of respiratory dysfunction in infants a rare consideration is a central etiology based on malformation of essential neuronal components of the brainstem.