Abstract
Central sleep apnea (CSA) is an important diagnostic and therapeutic challenge, which presents in a heterogeneous group of pathologies and comorbidities. Sometimes no cause is found to justify the presence of CSA, and it is classified as idiopathic. In this context, the performance of more specific complementary tests may reveal the presence of less frequent pathologies. We present a case of severe CSA in which brain MRI showed the presence of agenesis of the corpus callosum and dilatation of the third ventricle and lateral ventricles, without other associated malformations. It required treatment with adaptive servoventilation for its resolution. This association has only been described on one other occasion, so we believe that our case may contribute to focus attention on this infrequent cause of CSA.