Dentin dysplasia Type 1 (DD1) is an uncommon inherited condition marked by structural irregularities in dentin, leading to notable dental abnormalities. Clinically, patients typically present with generalized slight yellowish discoloration and tooth mobility, while radiographic examination often reveals a reduced pulp chamber with the absence of pulp stones, a hallmark feature of DD1. Treatment involves a multidisciplinary approach including extraction of affected teeth, direct sinus lift procedure bilaterally, implant placement, and subsequent fixed prosthesis placement. In a recent case, after six months, a patient demonstrated improved oral health-related quality of life with stabilized implant-supported prostheses providing functional and esthetic benefits. This emphasizes the importance of early diagnosis and intervention in managing DD1, underscoring the effectiveness of a multidisciplinary approach in enhancing oral function and esthetics. Further research is warranted to deepen our understanding of the genetic basis of this condition and develop targeted therapies.