Malignant tumors of visceral organs are a fundamental feature of familial cancer and paraneoplastic syndromes. In many instances, the presence of an internal and often occult malignancy may be forewarned by various external manifestations. Several of these findings are preferentially localized to the head and neck region, including the oral cavity proper. This places the dental practitioner in a unique position to detect these “markers” of occult neoplastic involvement. Because these markers may present before an established syndrome or cancer diagnosis, even representing the first expression of disease in some cases, early recognition by a dentist may lead to timely diagnosis and management of these cancer-associated syndromes.