Umbilical melanoma is an extremely rare and underreported disease with a complex underlying anatomy and the requirement for a tailored, specific approach. Embryological remnants in this region render this tumor more prone to metastatic spread via these structures. Inadequate treatment of this specific tumor can therefore result in a more devastating prognosis. This makes a more radical approach, compared to other melanoma, a necessity. Our aim is to provide an in-depth inside into the unique characteristics of this disease, the underlying anatomy and pathophysiology, and the required approach in order to be able to adequately manage this tumor. In this respect, we performed a detailed literature search for this rare disease as well as presented a case series from our tertiary center.