Tuberous sclerosis complex manifests predominantly as a neurocutaneous disorder. Lung involvement was considered rare. Lymphangioleiomyomatosis which occurs mainly in women of childbearing age is the major pulmonary disorder seen in tuberous sclerosis. Multifocal micronodular pneumocyte hyperplasia has also been described in tuberous sclerosis. The case of a 51-year old female diagnosed with tuberous sclerosis is described after she presented with progressive shortness of breath and was found to have interstitial lung disease. Tuberous sclerosis should be considered as a differential in patients with interstitial lung disease especially in association with cutaneous lesions.