A patient with basal cell naevus syndrome (Gorlin-Goltz Syndrome) is presented. Of note in the case was the extensive symmetrical tumour invasion of both external auditory canals requiring bilateral radical resection. The patient expired 14 months later, at which time the autopsy revealed widespread metastases to the pleura, diaphragm, pericardium, epicardium and myocardium. Although lung metastases have been reported in this syndrome, no cases have been reported of metastases to these sites.