Many inherited skin conditions are associated with underlying malignancies. The astute clinician will be able to recognize many of these cutaneous findings. The identification of these genetic syndromes with cutaneous findings, or genodermatoses, may allow early recognition of internal malignancies and the extension of these patients' life expectancy. Several of these syndromes have established screening and treatment protocols, which should be utilized in the care of affected patients. This article succinctly reviews the dermatologic, clinical, and laboratory findings associated with genodermatoses that have associated internal malignancies. Appropriate treatment and screening recommendations are explored.