Café-au-lait macules: occasional fatal sequels of benign pigmented lesions.

We report a case of sudden death in a 28-year-old male diagnosed with neurofibromatosis at the age of 6 years. The patient had multiple café-au-lait spots which, although being benign, were an ominous sign in this context. The immediate cause of death was intrathoracic neurofibroma causing compression that led to fracture of the thoracic vertebrae and laceration of the aorta with massive hemothorax.