Introduction: The Gorlin-Goltz Syndrome is rare but well-known multi-systemic syndrome. This case report highlights a Gorlin-Goltz Syndrome with left orbital and multiple facial basal cell carcinoma, calcified falx cerebri with cyst in the right mandible.
Case Report: A 47-year-old female presented with a 30 year history of slowly enlarging left periorbital mass eventually encroaching the left orbit. On gross examination, multiple nodular hyperpigmented masses with rolled edges on the entire left periorbital area with extension to the left orbit and globe and two 1x1 centimeter hyperpigmented nodular masses on both paranasal areas. The left globe was phthisical and enophthalmic with positive reverse relative afferent pupillary defect. The examination of the right eye, palms and phalanges was essentially normal. The patient underwent orbital exenteration, excision of left and right paranasal masses and enucleation of the right mandible cyst. The histopathology of the orbital and paranasal masses revealed basal cell carcinoma. Cranial computerized tomography revealed calcified falx cerebri. Transvaginal ultrasound revealed normal results. Chest radiograph showed no bifid ribs.
Conclusion: The importance of increasing awareness for early diagnosis and multi-disciplinary approach on treatment of this syndrome is of utmost importance to prevent life-threatening and vision-impairing complications of this disease. Frequent follow up and genetic counselling should be provided.

Keywords: Basal cell carcinoma, Calcified falx cerebri, Gorlin-Goltz