痣样基底细胞癌综合征(NBCCS)是一种常染色体显性遗传病,其基因外显率为97％,表现度不一,75％的患者早期可发生多发性基底细胞癌。此综合征有5个主要特征:1.多发性颌囊肿; 2.基底细胞癌;3.骨骼系统发育缺陷;4.手足点状小凹;5.异位钙化。此外,有某些器官系统的异常。本文报道一例3岁半女孩患双侧卵巢纤维瘤,在4岁时又发现基底细胞癌和手足多数小凹而诊断为NBCCS。1978 Barium-like basal cell carcinoma syndrome (NBCCS) is an autosomal dominant genetic disorder with a gene extroversion rate of 97%, varying degrees of expression, and 75% of patients may develop multiple basal cell carcinomas early. There are five main characteristics of this syndrome: 1. Multiple cysts; 2. Basal cell carcinoma; 3. Defective skeletal system development; 4. Spot-like pits; 5. Ectopic calcification. In addition, there are certain organ system abnormalities. This article reported a case of a 3-year-old girl with bilateral ovarian fibroids. At the age of 4 years, she also found basal cell carcinoma and most of the pits and hands in the diagnosis of NBCCS. 1978