Congenital dermal melanocytosis, or Mongolian spot, is the most common pigmentary dermatosis in newborns. It is frequently located in the lumbo-sacral region, although very infrequently it can be located in extra sacral areas. We present the case of a young infant with an atypical Mongolian spot on the trunk and extremities, which undoubtedly represents a diagnostic challenge for primary care physicians, since this presentation is very rare and may be associated with other clinical situations that should be suspect.
