Recently, we have experienced significant numbers of patients diagnosed with non-specific interstitial pneumonia (NSIP) by open lung biopsy or video-assisted thoracoscopic surgery. The purpose of this work was to describe the clinical features of patients with collagen vascular disorders (CVD) presenting NSIP in the absence of systemic involvement. This study also involved a retrospective review of patients with CVD presenting clinical and pathological evidence of NSIP in the absence of systemic manifestations of CVD. We found seven patients (six from our experience and one from literature review) with histologically proven NSIP who later developed typical CVD more than 6 months after the first presentation of NSIP. In these cases, it was difficult to speculate the development of CVD at the point of first presentation. Therefore, association of CVD should be considered in patients with NSIP even in the absence of classical systemic involvement.