BACKGROUND: Oral cavity neurovascular hamartomas are extremely rare, with unknown prevalence, possibly due to their rarity, unrecognition or under-report. We describe a 6-year-old female patient with this uncommon lesion and review existing literature. RESULTS: The patient presented with asymptomatic papular lesions, which increased in size and number since birth. First, one of the papular lesions on the lower lip was excised for histopathologic study, revealing the unexpected diagnosis. DISCUSSION: Oral neurovascular hamartomas are unusual and challenging lesions to diagnose. Even though their benign nature, the need of differential diagnosis with other oral lesions makes necessary their histopathological study and confirmation. CONCLUSIONS: Even oral neurovascular hamartoma is an uncommon entity, surgeons and oral pathologist should be aware of this entity in order to distinguish it from other lesions. Besides, it is not clearly related to rhabdoid tumors as other authors have stablished previously, being an independent entity with great prognosis for the patient.