Most periapical lesions are represented by inflammatory cysts, granulomas, abscesses or fibrous scars. These inflammatory conditions are often termed "endodontic lesions" because pulpal necrosis is the initiating event in their pathogenesis. Although rare, other clinically confusing periapical lesions have been extensively documented in numerous case reports and short case series. These lesions represent a wide range of pathosis, including various developmental cysts, infections, benign but locally aggressive lesions, and malignancies. The literature describing these lesions and the value of a histopathologic examination in diagnosis is reviewed.