Pilomatricoma, also known as calcifying epithelioma of Malherbe or pilomatrixoma, is a small benign tumor of hair cortex cell origin. A molecular defect in beta-catenin is present in the lesions. Pilomatricoma tends to arise during early childhood as solitary, asymptomatic tumors. Although usually sporadic in incidence, familial cases of pilomatricomas have been reported. Additionally, multiple pilomatricomas have been observed in patients with myotonic dystrophy, Gardner syndrome, Rubinstein-Taybi syndrome, constitutional mismatch repair deficiency, Kabuki syndrome, and trisomy 9, and in patients with PENS (papular epidermal nevus with "skyline" basal cell layer).