ABSTRACTParasitic infections rarely involve the oral and maxillofacial regions and pose a diagnostic challenge when they do. Hydatid cysts are parasitic cysts caused byEchinococcus granulosus. Intraosseous involvement is observed in 3% of cases, of which only 2-6% are in the maxillofacial region. A scientific literature search revealed only seven cases involving the mandible. We report a rare case in a 16-year-old female patient who presented with facial asymmetry and well-defined radiolucency of the ramus. Our findings will help in understanding the diagnostic issues caused by non-specific presentation and difficulties in suspecting such a rare diagnosis as echinococcosis of the oral or maxillofacial region. A thorough systemic investigation is essential as 20-30% of these cases show multiorgan involvement.