Bibliographic evidence regarding oligodontia was reviewed to extract information necessary for a systematic review for a prosthodontic approach to management. Syndromic oligodontia, appearing as a symptom of many syndromes, was distinguished from isolated, or non-syndromic, which is an independent trait. Although a rare disorder, oligodontia has always been considerably researched, especially concerning its prevalence and genetic background. Non-syndromic oligodontia has been associated with the presence of small and misshapen natural teeth, orofacial clefting and reduced saliva secretion. A typical maxillofacial morphology has also been reported, which seems to result from the lack of dental and functional compensation and not from an altered growth pattern. Syndromic oligodontias also exhibit the above basic features but are complicated by each syndrome's specific characteristics. Prosthodontic treatment of individuals with oligodontia must anticipate the dental and oral clinical characteristics and provide with continuing support and preservation of proper maxillofacial relationships.