Discoid lupus erythematosus (DLE) is uncommon in childhood. Less than 2% of patients with DLE develop the disease before 10 years of age.We present eight cases of childhood DLE with onset before age 10 years: four black boys with cutaneous DLE, three black girls ages 7, 2, and 6 years at onset, who developed systemic lupus erythematosus at ages 12, 9, and 8 years, respectively, and a 10-year-old Hispanic boy who had a systemic flare at the age of 20 years.A review of the 16 published cases of childhood DLE reveals that it is similar to its adult counterpart in its presentation and chronic course. However, several important differences are noted: a lack of female predominance, a low incidence of photosensitivity, and frequent progression to systemic lupus erythematosus at an early age. A discussion of the management of DLE in children is also presented.