Many oral lesions of the oral cavity are a result of anatomical variants and developmental anomalies. Most of these anomalies arise at the 5–8 weeks of intrauterine life and are classified as deformities (anomalies in shape) and/or malformations (anomalies in formation). Appropriate clinical diagnoses of these anomalies lead to treatment when indicated. Diagnosis may be challenging, and developing a list of differential diagnoses of these anatomical variants and developmental anomalies may be beneficial.