A 36-year-old woman with a linear and unilateral cutaneous hamartoma is described. The lesions consisted of hypopigmented macules that coalesced into a linear plaque that involved only the left side of her body. Within this plaque were several pearly papules and an ulcerated area that clinically resembled basal cell carcinomas. Histologic features of the hypopigmented linear plaque included multiple and multifocal basaloid proliferations in the papillary and mid dermis. The isolated pearly papules showed the typical findings of basal cell carcinoma.