Xeroderma pigmentosum (XP) is a genodermatosis characterized by pigmentary abnormalities, solar skin damage and cutaneous malignancies in the sun-exposed areas. The various skin tumors seen in XP include basal and squamous cell carcinomas, malignant melanomas and less commonly keratoacanthomas, angiomas, fibromas and sarcomas. Pilomatricoma is a benign neoplasm with differentiation towards the hair matrix and has been reported in a single case in association with XP . We report a new case for such an occurrence. A 9-year-old boy, a known case of familial XP presented with one nodular lesion, on the nose measuring 1 cm of four months duration (Figure 1). It was an erythematous, rounded nodule, firm in consistency and showed no overlying skin changes. In the dermoscopic view, we can find irregular white structures, some reddish homogeneous areas. There was no lymphadenopathy. This lesion was excised and sent for histopathological study and the diagnosis of pilomatricoma was made (Figure 2).