Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant trait, which depicted presence of numerous basal cell carcinoma in conjunction with multiorgan abnormalities. This syndrome may be diagnosed early by a dentist by routine radiographic exams in the first decade of life, since the keratocystic odontogenic tumour are usually one of the first manifestations of the syndrome. This article includes a case report of the GGS with regard to its history, incidence, etiology, features, investigations, diagnostic criteria, keratocystic odontogenic tumour and treatment modalities.

Maxillary sinus odontogenic keratocyst (OKC) is very rare and occupies less than 1% of the total OKC cases reported in the literature. OKCs have characteristic features that are unique compared to other cysts of the maxillofacial region. Considering their peculiar behaviour, varied origin, debated development, discourse treatment modalities, and high recurrence rate, OKCs have been a subject of interest for various oral surgeons and pathologists globally. This case report presents an unusual case of invasive maxillary sinus OKC into the orbital floor, pterygoid plates, and hard palate in a 30-year-old female. The case report confers that cystic maxillary sinus lesions should always be treated very extensively irrespective of the nature of the lesion as the site makes it highly susceptible to secondary infection and recurrence. The case also establishes a set of imaging modalities and specific treatment approaches to be followed for maxillary sinus OKC based on the literature of all the previous cases reported.

Gorlin-Goltz syndrome is an autosomal dominant inherited condition comprising the principle triad of basal cell carcinomas, multiple jaw keratocysts, and skeletal anomalies. The presence of jaw cysts are the early diagnostic feature of this syndrome, and this can be incidentally identified by routine radiographs. A patient presented with signs and symptoms of Gorlin-Goltz syndrome to us in her early stages.

Primary intraosseous squamous cell carcinoma (PIOSCC) derived from an odontogenic keratocyst (OKC) is a rare malignant neoplasm of the jaws, which is locally aggressive with quite poor prognosis. The incidence of carcinomas arising in odontogenic cysts was reported to be approximately 1-2/1000. The number of well-documented cases of PIOSCC ex OKC is extremely small; hence, no sufficient incidence data are available in the literature. Overall, the survival rate of an individual, which is a period of two years, is very poor, and this can be attributed to the delayed diagnosis. But knowledge of the histopathological and immunohistological features of PIOSCC allows accurate and early diagnosis of the lesion so that an early and appropriate treatment can be instituted for better prognosis. The following report describes an extremely rare case of PIOSCC of the mandible derived from an OKC in a 20-year-old female patient.