Sickle-cell disease is a frequent hemoglobinopathy which represents a major public health problem as a result of the continuous migration of populations. The clinical manifestations are chronic hemolytic anemia, vaso-occlusive crisis and infections; however, the involvement of the head and neck region remains poorly reported. Particularly, we do not know the prevalence of the odontostomatological manifestations.In this study, we reviewed the most recent literature on the topic. We have classified the alterations of the orofacial sphere into four categories: oral manifestations, bone changes, infections and various. We have described the main clinical manifestations of each one, along with their incidence and prevalence, trying to identify the pathophysiological mechanisms, correlations with vaso-occlusive crisis and possible therapeutic strategies. Several odontostomatological manifestations are described in patients with sickle cell disease. Some are typical, while others still need to be investigated, as for example the relationship between oral triggers and vaso-occlusive crisis.