The cutaneous sclerosis, defined as a pathological increase of the elements composing the dermis or hypodermis, results in thickening and hardening of the skin. This cutaneous manifestation is often associated with systemic sclerosis. However, it is also found in other less well-known syndromes that are highly heterogeneous in their clinical presentations. This article reviews the etiologies, clinical presentations, and possible treatments of these rare pathologies.