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Gorlin-Goltz syndrome is an uncommonly found rare disease which shows various possible diverse manifestations of multisystem anomalies, high degree penetration rate with variable expressiveness at least on three body systems. Cutaneous, skeletal, ophthalmological, neurological and reproductive systems are commonly affected in this syndrome. Multiple keratocysts of the jaws are the frequently developed early abnormality than any other. Hereditary autosomal dominant trait is explained as the causative factor for the development of the syndrome. In this case two major and seven minor anomalies were registered. The anomalies found in the patient were not categorized as serious life-threatening abnormalities excepting the multiple keratocysts which were treated by enucleation, curettage and Carnoy's solution application. An incidental detection of Gorlin-Goltz syndrome with multisystem anomalies of a 14-year-old female patient, who had reported for the swelling in the left body of the mandible, is described in this article.

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TMJ Ankylosis is a devastating condition occurs secondary to trauma. Patient with Tmjankylosis not only have difficulty in mouth opening but also have marked facial deformity. Various treatment modalities are available for the management of this deformity like release of ankylotic mass reconstruction;, primary distraction Osteogenesis and secondarily release of ankylotic mass.The aim of this study was to evaluate the correction of facial deformity using distraction osteogenesis principlesIn the present study total of five patients were selected. In all the patients, in first stage TMJ ankylotic mass were removed. In a second stage distraction Osteogenesis were performed on the affected side for the correction of facial deformity. Distraction Osteogenesis after release of TMJ ankylosis is a feasible and reliable technique for the correction of secondary mandibular deformity.