Oligodontia is a rare dental anomaly with a prevalence of 0.3% in permanent teeth and much less frequency in the primary dentition. Familial oligodontia represents an absence of varying numbers of primary and/or secondary teeth as an isolated trait. It is a complex and multifactorial condition. Many explanations-evolutionary, genetic, and environmental-have been proposed as the etiology. Simultaneous with oligodontia are often the different positional changes of the existing teeth, their morphology, size, and growth disturbances of the maxillofacial skeleton. Early recognition is vital to provide adequate treatment and prevent squeal. Multidisciplinary referral or consultation is thus important in treatment planning to improve function and esthetics. The present paper reports a rare case of familial oligodontia associated with multiple dense invaginatus and microdontia.