Basal cell nevus syndrome (BCCS) is an autosomal-dominant disease causing the development of multiple basal cell carcinomas, together with other multiorgan abnormalities. Multiple BCCs usually manifest early in life, up to several hundreds of elements, along with black or red palmoplantar pits. Acrochordon-like BCCs and small acral BCCs (basaloid follicular hamartomas) have also been reported. Dermatoscopy of BCCs shows the classical features described in sporadic BCC (wheel areas, blue-grey globules, large ovoid nests, ulceration, erosions, arborizing vessels, etc.). Basaloid follicular hamartomas present blue-grey globules in the absence of vascular structures. Black palmoplantar pits appear as depressed black-brown lesions without vascular structure on a yellowish background, while the red variant shows irregular-shaped and slightly depressed lesions with a yellow-pinkish background, whitish borders, and arborizing vascular structures.