We present in this report a case of an infant girl who was born with natal teeth along with an ulcerated rapidly involuting congenital hemangioma (RICH) on her thigh. RICH is a benign vascular tumor that is more common than other congenital hemangioma subtypes. Natal teeth are a birth anomaly that occurs in isolated form or in association with different syndromes like Ellis-van Creveld, Pierre Robin, Soto and Hallerman-Streiff. We have performed an intensive literature search to determine if there is a link between natal teeth and RICH, but no article has described any association thus far. It remains unclear whether this is a mere coincidence or whether there are common genetic factors that play a role in benign tumor growth and eruption of premature teeth. Further reports on the co-occurrence may shed light on possible common risk factors contributing to natal teeth and congenital hemangioma.