ABSTRACT Background Nevus of Ito and Mongolian spots are distinct clinical presentations of patchy dermal melanocytosis, characterized by similar dermatological manifestations that can pose diagnostic difficulties for clinicians. Aim This review aims to consolidate current understanding and research advancements on these conditions to facilitate clinical diagnosis, differential diagnosis, and management. Methods A comprehensive search of databases including PubMed and Google Scholar was conducted, along with an analysis of pertinent literature retrieved from reference lists spanning nearly four decades. Results Epidemiological, clinical, and pathological profiles exhibit nuanced differences between the two conditions, with unique expressions under electron microscopy and the regression possibility. It is noteworthy that most Mongolian spots naturally fade with advancing age, in contrast to nevus of Ito, which persist and may potentially evolve into malignant lesions. While picosecond laser treatment has shown greater efficacy than nanosecond lasers, the lower‐energy approach holds particular promise in pediatric cases. The therapeutic landscape for patchy dermal melanocytosis is evolving, shifting from selective photothermal action to photomechanical or subcellular photothermal modalities. Conclusion This review underscores the importance of meticulous clinical assessment and the potential of innovative therapeutic approaches in managing these conditions.