The epidemiology of the dental disease in sickle cell anemia is not adequately described. The aim of this work is either the evaluation of literature on the topic, or to describe if the disease itself predisposes to a dental pathology, and eventually also if the latter can influence its course. We selected many cases and reviews in order to identify the dental diseases related to sickle cell anemia. Caries and periodontitis are not directly caused by disease. However, an inflammatory state caused by a dental infection can trigger or precipitate a sickle crisis. Malocclusion angle class II is particularly frequent, as a result of bone facial changes and/or related to muscle imbalance. Temporomandibular joint disorder is possible. Asymptomatic pulp necrosis is due to the sickling that causes vasocclusion within microcirculation of the dental pulp. Large population-based studies are needed in order to clarify the dental involvement in this disease. A strict collaboration between the dentist and the hematologist is essential.