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Introduction: Granulomatosis with polyangiitis (GPA), is a rare systemic disease that if left untreated, it may lead to death within 6-12 months. This case report describes a 15-year-old female with a 14-month history of epiphora, nasal breathing difficulties, headaches, and jaw pain. The patient reported having various medical procedures attempted to address her symptoms. The classical presentation of strawberry gingivitis led to the diagnosis of GPA. Based on a multi-language search, this is the first reported case, that GPA is mistaken as Temporomandibular Joint Disorders (TMD). Case Presentation: A 15-year-old female with nearly 14 months of numerous complaints, including headaches and jaw pain, was referred for an orofacial pain (OFP) consultation. The patient had completed a dental examination and was prescribed chlorhexidine for gingivitis control. The OFP examination was not consistent with signs and symptoms of TMD. However, the gingival appearance of strawberry gingivitis was suggestive of GPA. A rheumatologist confirmed the diagnosis of GPA based on the clinical interview, serology testing, and dental findings. A course of rituximab and corticosteroids, and regular dental cleanings were recommended. A 4-month follow-up visit demonstrated complete resolution of her jaw pain and headaches. Gingival tissue appeared normal. Conclusion: This case emphasizes the need for familiarity with systemic diseases that can present oral manifestations. It also stresses the importance for dental professionals to be knowledgeable of differential diagnoses for TMD, headaches, and autoimmune disorders. K E Y W O R D Sgingival hyperplasia, oral medicine, pain, pathology -oral, temporomandibular disorders, strawberry gingivitis Why is this case new information?• This is believed to be the first published case mistaking GPA as TMD.• This is a case in which a multidisciplinary approach and management were keys for a successful treatment outcome.What are the keys to successful management of this case?• Obtain a thorough clinical history.• Know the oral manifestation of systemic diseases.• Know differential diagnosis of TMD and headaches.

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