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Basal cell nevus syndrome Congenital hydrocephaly Abstract A case of basal cell nevus syndrome or Gorlin’s syndrome is reported in a new-born. The skin condition is associated with congenital hydrocephaly and skeletal malformations. To our knowledge, this is the first case of basal cell nevus syndrome with skin tumors present at birth and localized on the fingers K. Meyvisch, Department of Dermatology, University of Brussels, B-1200 Brussels (Belgium) Basal cell nevus syndrome is an autosomal dominant inherited condition although some sporadic cases have been reported [1]. It was first described by Gorlin and Goltz in 1960 [2] and characterized by five major components: multiple basal cell carcinomas appearing at an early age; jaw cysts; skeletal anomalies, especially of the ribs, spine, skull and metacar-pals; soft tissue calcification, and palmar and plantar pits. A variety of other, less common defects have been described [3], among which are neurologic abnormalities. We saw a 5-month-old boy with basal cell nevus syndrome, associated with congenital hydrocephaly. Skin lesions were present since birth and localized on the fingers, whereas they usually appear at puberty [4] and arise on the face and trunk. smooth-surfaced rounded elevated papules, flesh-colored or pig-mented and varying in size from 1 to 6 mm in diameter, localized on the lateral sides of the fingers of both hands (fig. 1). Pits are absent. Further clinical examination reveals a particular facies with ocular hypertelorism, frontal bossing (fig. 2) and macrocephaly. Routine laboratory investigations were within normal limits. The karyotype is normal. A skin biopsy is performed, and histological as well as ultrastructural examination confirmed the presence of a superficial and middermal nodule, which consists of basophilic cells which are arranged in characteristic palisades (fig. 3). This confirms the diagnosis of basal cell carcinoma. Ophthalmological examination cannot reveal any anomaly. A radiological checkup shows a fourth bifid rib on the right side and a spina bifida occulta (D2D6). There are no jaw cysts, no calcification of the falx cerebri nor brachymetacarpalism. Discussion