Juvenile dermatomyositis (JDM) is an uncommon inflammatory myopathy with characteristic cutaneous manifestations. Certain clinical features such as cutaneous calcification, lipoatrophy, and generalized hypertrichosis can occur in JDM but are uncommon in the adult form of the disease. We report a young girl who presented with dramatic infrapatellar hypertrichosis along with more classic clinical signs of JDM, including heliotrope periorbital eruption, photosensitivity, and profound proximal muscle weakness. The clinical course was favorable, with improvement of cutaneous and muscular abnormalities after corticosteroid treatment.