RP-ILD is frequently associated with anti-MDA5 and anti-ARS antibodies and typically predicts a poor prognosis [4]. We present an extraordinary case of isolated anti-Mi-2-related RP-ILD occurring without muscle or skin manifestations, illustrating the diagnostic complexity and extending the clinical spectrum of IIM [5,6]. This evidence concerns the gene IFIH1 and retinitis pigmentosa 1.