We describe the case of a 28 years old caucasian woman with tuberous sclerosis diagnosed at 23 when she underwent nephrectomy for a left renal angiomyolipoma with massive hematuria. She had adenoma but she presented on mental deficit and there was no record of seizures. Familiar history was negative except for the presence of adenoma sebaceum in her father, aged 63, and in her only daughter, aged 10. Five years after nephrectomy she was admitted to our hospital because of exertional dyspnoea and haemoptysis. Pulmonary involvement was confirmed by chest X-rays and CT scans.