Introduction: Nevoid BCC syndrome (Gorline syndrome) is a familial disorder with autosomal dominant inheritense. This syndrome is combination of multiple BCC that occurs at an early age, characteristic faces with: frontal bossing, broad nasal bridge and hypertelorism, jaw cysts, palmoplanter pitting, macrocephaly, skeletal and spinal anomalies include bifid ribes, cervical rib and kyphoscoliosis, CNS abnormality include corpus callusom disgenesia , falx cerebri calcification(at early age) and mental deficiency.

Case Report: This case was a 25-years-old female presented with multiple and progressive skin lesions with different size in neck, upper trunk and axilla (multiple BCC), palmoplantar pitting, jaw cyst, cervical rib, bifid rib and liver haemangioma.

Conclusion: With combination of clinical feature, histopathological reports of skin lesions and radiological reports of mandibular cyst and ribs anomalies, this case was diagnosed as nevoid BCC syndrome.