RESUMO
A basal cell nevus syndrome, known as the Gorlin syndrome, is a rare autosomal dominant disorder, occuring especially in the oriental population. It is a complex hamartomatous/neoplastic syndrome with multisystemic manifestations involving the five major features: (1) multiple basal cell nevi, occasionally basal cell carcinoma usually seen at an early age; (2) multiple jaw cysts; (3) skeletal abnormalities of ribs, skull, and spine; (4) ectopic calcification; (5) palmar and plantar pits. Because patients are predisposed to this disorder prior to basal cell carcinoma of the skin, ovarian fibroma, and medulloblastoma, the most important aspects of management is frequent examination and early treatment of small tumors. We report here about a 16-year- child with odontogenic maxillary sinusitis, who has developmental multisystemic disorders which coincided with the basal cell nevus syndrome. The patient underwent a sinus operation and now is under a cautious follow-up.