The blue rubber nevus syndrome describes a clinical entity that presents with cutaneous and visceral hemangiomas, particularly those found in the gastrointestinal tract. These lesions may appear at birth or in early childhood and can increase in size and frequency with age. Oral cavity hemangiomas occur in 59% to 64% of cases. Successful diagnosis requires a high index of suspicion, and treatment is symptomatic. This report discusses a unique presentation and a review of the literature.