Tuberous sclerosis is a very infrequent hereditary disease, usually defined by the triad of epilepsy, mental retardation and adenoma sebaceum, although alterations in other organs can exist. Tuberous sclerosis in its association with pregnancy has seldom been described in the literature. One such case is reported here; that of a 24-yr-old woman whose pulmonary involvement constitutes its differentiating characteristic. We make special mention of the improvement of the respiratory function, without any modification of the renal function, in contrast with other previously published cases. She gave birth spontaneously by vaginal via. Finally, we underline the importance of prenatal diagnosis and genetic advice for these patients.