The combination of infraoccluded primary molars and agenesis of the permanent successors is rare. This condition progresses with increased severity since the primary teeth do not usually exfoliate spontaneously. The clinical examination of an 11-year-old girl revealed 4 submerged primary second molars, agenesis of all second premolars, and a peg-shaped maxillary lateral incisor. Treatment in this case was surgical extraction of the submerged teeth and placement of removable appliances to maintain space and vertical dimension. Keywords: agenesis, ankylosis, infraocclusion, peg-shaped tooth, primary teeth, removable appliance, surgical treatment