Nevus spilus is a term in the literature used for a benign pigmented cutaneous lesion, occurring shortly after birth or in the early stages of infancy. The lesion itself is very distinguishable in most cases with numerous small papules or macules on a "café au lait" background pigmentation. It can be seen on different parts of the body with predominance in the areas of the extremities. Although benign, in some cases malignant transformation can occur. Malignant melanoma arising within a nevus spilus lesion is not so unlikely anymore. We report a 36-year-old female patient with a thin cutaneous melanoma developing within a congenital nevus spilus lesion successfully treated with surgery. The complete surgical removal of melanoma and nevus spilus ensures the absence of recurrences as well as the need for follow-up of patients. This surgical approach should be discussed with patients, and clinicians' recommendations depend on 1) the morphology of the lesion, 2) the size of the lesion, 3) the localization of the lesion, and last but not least : the presence or absence of stigmatization in the patients themselves. Our main focus in this article will be on the importance of an early diagnosis and eradication of such lesions while reviewing the existing literature.