Abstract: Nose is one of the most commonly associated defect in the trauma involving craniofacial region. The resulting aesthetic and structural defect causes facial disfigurement affecting the overall personality of the patient.The defect not only affects the individual with impaired functionality but also psychologically. Hence, the treatment involves multidisciplinary approach in the management of cases. Background: The purpose of this Case report primarily focuses on the prosthetic rehabilitation as treatment modality in management of nasal defect in Pigmentary Mosaicism. Case Presentation: The 14 year old patient reported with the complain of missing central incisor and nasal defect. Rehabilitation of the single missing was done with removable partial denture. Patient was diagnosed with a rare disorder of Pigmentary Mosaicism with nasal defect as the associated deformity which also represents with cutaneous and the extra-cutaneous involvement. Along with the nasal defect the patient also manifested with Hyper and hypopigmented patches on the skin overall on the body surface in mosaic pattern, Which was treated simultaneously.