Jacob's disease is a rare condition that consists of the formation of a pseudo joint structure between the mandibular coronoid process and the zygomatic bone, resulting in limited mouth opening. The disease is difficult to diagnose and etiology is uncertain. This article describes a clinical case of a 6-year-old child with limited mouth opening, which resulted in the formation of a temporomandibular ankylosis leading to complete immobility due to delayed diagnosis and treatment of the disease. A review of this pathology and the most precise imaging exams are discussed for the early and differential diagnosis of the disease.
 Descriptors: Ankylosis; Temporomandibular Joint; Temporomandibular Joint Disorders.
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