This report describes the understudied co-occurrence of temporomandibular joint ankylosis (TMJA) and extrahepatic portal vein obstruction (EHPVO), exploring a shared pathway involving hypercoagulability. TMJA is an acquired pathology where joint surfaces fuse, causing restricted mouth opening and facial asymmetry. Globally, TMJA is prevalent among 1.5 to 5 patients/million, with a higher incidence in developing countries. While trauma and infections often cause TMJA, the pathogenesis remains unclear in many cases. Recent literature notes a link between TMJA and EHPVO, a noncirrhotic vascular disorder causing portal hypertension and upper gastrointestinal bleeding in children. Prothrombotic disorders such as protein C and S deficiency may contribute to EHPVO, mirroring TMJA's association with hypercoagulability. This report focuses on an 11-year-old female diagnosed with TMJA, accompanied by a history of ear infection and concurrent EHPVO. We further presented clinical observations, surgical interventions, and outcomes alongside a literature review to understand the probable connection between EHPVO and TMJA.