Schizencephaly is a rare CNS malformation consisting of a full-thickness CSF-filled parenchymal cleft lined by grey matter. Schizencephaly can be diagnosed on prenatal ultrasound but requires magnetic resonance imaging (MRI) for more specific diagnostic differentiation from other cerebrospinal fluid (CSF)-containing structures as well as for detection of associated features.

Adenomatoid odontogenic tumor is a rare benign odontogenic tumor comprising of 3% to 7% of all Odontogenic tumors. It is usually asymptomatic, well circumscribed, slow growing tumor associated often with impacted canine and discovered on routine radiographs. Microscopically this lesion shows a variety of patterns forming sheets, strands, nest or rosette-like structures with ductal pattern with central space surrounded by single layer of cuboidal to columnar epithelial cells with a rim of eosinophilic coagulum which is the characteristic feature of these tumors. In this article we present two extrafollicular cases of this rare tumor occurring in a male and a female patient in the maxillary anterior region with both symptomatic and asymptomatic presentation. Both the tumors were not associated with any impacted tooth. Adenomatiod Odontogenic tumor was confirmed based on histopathology.