Hemifacial hyperplasia is a rare developmental anomaly characterized by marked unilateral facial tissues. It involves orofacial soft tissues, bones of the face, and teeth. The cause remains ambiguous although several predisposing factors have been reported. A case report of a 32-year-old girl with unilateral hemifacial enlargement, pain in temporomandibular joint, and limited mouth opening associated is presented to highlight the clinical and imaging findings and to discuss the differential diagnosis.