The Mongolian spot is a congenital, blue-gray color with a variety of shapes and sizes, mostly located in the lumbosacral area, commonly observed in Asian and African infants but rare in Caucasians. A diagnosis is based on the clinical morphology. A histopathological examination of the lesions is required when in doubt. Mongolian spots are often present at birth, usually disappear during childhood, and are generally unnoticeable after one to four years. On the other hand, these spots persist for a long time in approximately 3%-4% of Asian populations. Darker pigmented spots located in the extrasacral area are called ectopic Mongolian spots (previously known as aberrant Mongolian spots). These are more likely to persist than the typical sacral Mongolian spots. They have a benign course, but patients with ectopic Mongolian spots in exposed areas may experience psychological distress owing to their cosmetic appearance. Herein, ectopic Mongolian spots are described in terms of their epidemiology, pathogenesis, clinical features, pathological features, and treatment.