Lesions in the skull base may present difficulties in diagnosis primarily because the access needs to be invasive and one has to rely on imaging that may still be misleading. The case presented here illustrates this example in that the patient had abnormalities on computed tomography (CT) and magnetic resonance image (MRI) scans quite convincing of malignancy but which with time proved, essentially through clinical judgment, to be skull base osteomyelitis secondary to malignant external otitis (OME).

Familial autosomal dominant cylindromatosis (FADC, turban tumour syndrome, Brooke-Spiegler-syndrome and many more, MacKusick catalogue numbers 123850, 313100) is a rare hereditary disease usually presenting in the second or third decade. With female preponderance dermal cylindromas predominantly arise in hairy areas of the body with approximately 90 per cent on the head and neck. Transformation to malignancy seems to be scarce. Although cylindromas of the skin resemble basal cell adenomas of the salivary gland, there is usually no salivary gland involvement. On the other hand, patients with basal cell adenomas of a salivary gland usually do not show dermal lesions. We report one of the rare cases of FADC combined with multiple basal cell adenomas of the parotid glands and present a review of the literature.