We report three Japanese children with Mycobacterium avium infection of the skin who also developed lichen scrofulosorum, a previously undescribed association. They were healthy except for the presence of several noduloulcerative lesions associated with multiple asymptomatic papules on the trunk and extremities. Histology of the ulcerative lesions showed features of mixed-cell granuloma, whereas the papular lesions showed features consistent with lichen scrofulosorum. M. avium was identified by polymerase chain reaction-aided DNA-DNA hybridization analysis in specimens obtained from the noduloulcerative lesions. Both the noduloulcerative and the papular lesions responded well to combination chemotherapy consisting of antituberculous agents and antibiotics.