Introduction: in 1952, Neligan and Strange first described a rare phenomenon, which occurs in the neonatal period and is characterized by a sudden change in skin color, with a straight line dividing half of the body as if it had been “drawn with a pencil and ruler” running down the midline, with one half of the body erythematous and the other pallor. Objective: to present literature related to the Harlequin color change phenomenon and its different clinical presentations. Methods: a non-systematic review of the available literature was conducted using key terms such as “Harlequin Color Change”, “Harlequin Newborn”, “Harlequin Color Change NOT Syndrome NOT ichthyosis” using other Boolean operators such as “AND” and “OR”, both in English and Spanish, in the PubMed, ScienceDirect, and Google Academics databases. Results: Harlequin Color Change is characterized by an appearance of skin divided into two distinctly colored areas. Its etiology is unknown but thought to be caused by immaturity of hypothalamic regulation of peripheral vascular tone. It affects approximately 10% of healthy neonates. Conclusion: Harlequin Color Change is a benign, idiopathic, and self-limited condition that does not need treatment. The physician must recognize this phenomenon, which has a variety of color change patterns. It is essential to inform parents of this harmless and temporary skin condition to prevent needless testing and treatment. Not to be confused with ichthyosis Harlequin.