RESUMO
Dermoid cyst with dermal sinus in occipital region is extremely rare occurrencl and still rarer in asterion region. We report a case of dermoid cyst with dermal sinus at the asterion probably the second case of world literature and discuss the findings. 16 year-old female patient with mental retardation and inability to speak since childhood presented with history of progressive increasing swelling since childhood over the left side of occipital region. Local examination and investigations were suggestive of extracranial cystic lesion. The lesion was excised completely. When excision of such lesions is contemplated, careful attention must be paid during exploration. Failure to recognize intracranial communication in such lesions may result in fatal consequences. Pre-operative suspicion of such a connection helped us to identify the underlying defect intra-operatively and facilitated safe excision