Patient: Female, 14Final Diagnosis: Rhabdomyomatous mesenchymal hamartoma (RMH) presenting with trigeminal neuralgia (TN)Symptoms: Facial Swelling • numbness • painMedication: —Clinical Procedure: Surgical removal of RMHSpecialty: OtolaryngologyObjective:Unusual clinical courseBackground:Rhabdomyomatous mesenchymal hamartoma (RMH) is a benign, potentially pigmented lesion that occurs in the head and neck region. It generally consists of haphazardly arranged skeletal muscle with adipose tissue, blood vessels, collagen and nerve fibers and is largely asymptomatic. Trigeminal neuralgia is pain due to compression of the trigeminal nerve. TN may be idiopathic or associated with lesion-mediated compression.Case Report:We describe the case of a 14-year-old female presenting with trigeminal neuralgia (TN) associated with RMH. On initial consultation, the patient presented with a history of right-sided lower facial swelling, numbness, and pain. Evaluation by various specialists confirmed TN. Surgical resection of the lesion resolved the condition and pathology confirmed RMH.Conclusions:This is the first case report demonstrating RMH-mediated TN. Surgical resection of the RMH is a safe management approach for this diagnosis.