Multiple odontogenic keratocysts could be linked to different conditions. Then, to achieve the correct diagnosis whether their presence is associated to a syndrome, some criteria must be followed. The present study aims to report a case of a 21 years-old male patient whose was referred with several radiolucent lesion on the maxilla-mandibular complex. The lesions were biopsied and the diagnosis of the histological exam hypothesized as odontogenic keratocyst. After complete evaluation, others abnormalities were also found such as calcification of falx cerebri, palmar and plantar pits, and multiple basal cell on feet. The patient was diagnosed with Gorlin-Goltz syndrome and was referred to other medical specialties to adequate follow-up. Dental surgeon represents an important role on correct diagnosis of the Gorlin-Goltz syndrome and could avoid further complications.