角化囊肿综合征是一种较为罕见的常染色体显性遗传疾病,以颌骨多发性角化囊肿为主要临床表现,可同时伴发分叉肋、大脑镰钙化、小脑幕钙化等骨骼异常,当同时伴发痣样基底细胞癌时,又称痣样基底细胞癌综合征。本文报道1例角化囊肿综合征病例,并对其临床、病理及治疗进行了讨论。 Keratocystis syndrome is a rare autosomal dominant genetic disease, multiple keratocysts of the jaw as the main clinical manifestations, can be accompanied by bifurcation ribs, cerebral falx calcification, calcification and other cerebellar calcifications and other skeletal abnormalities, When accompanied by nevus-like basal cell carcinoma, also known as nevus-like basal cell carcinoma syndrome. This article reports a case of keratoconjunctivitis syndrome, and its clinical, pathological and treatment were discussed.