Discoid lupus erythematosus (DLE) is a rare disorder in childhood, with 22 cases reported in the English-language literature. Less than 2% of patients with DLE have an onset before 10 years of age. We describe two children with DLE and lupus profundus with an onset of the disease at the ages of 11 and 15 years and focus on likely histopathologic differences between DLE in children and in adults. Histopathologic characteristics for childhood DLE might be an intense periadnexal and perivascular infiltrate extending into the interstitium and subcutaneous tissue consisting of lymphocytes, histiocytes, eosinophilic granulocytes, and plasma cells and lacking epidermal atrophy. The diagnosis of DLE in our two patients was established by clinicopathologic correlation based on clinical presentation, histologic and immunofluorescent findings in skin biopsy specimens, and the absence of clinical and laboratory evidence of systemic involvement. Therapy with antimalarials resulted in reduction of the skin lesions, but in one patient severe lipoatrophy occurred.