A pilomatricoma, or Malherbe's calcifying epithelioma, is an uncommon tumor originating from hair matrix cells. It is clinically characterized by a solitary, firm nodule. As the skin overlying the pilomatricoma may change in color and texture, its clinical presentation can vary. We report an unusual case of pilomatricoma with associated anetoderma on the lower extremity of a 12-year-old girl. Histology revealed a thinned dermis replaced by myxomatous tissue between the surface and a deep-seated tumoral mass. This mass is formed of irregular islands of basaloid cells, shadow cells, calcified areas and discrete inflammatory and foreign-body reactions surrounding it. Anetodermic cutaneous changes may occur in pilomatricomas without histological evidence of triggering factors.