INTRODUCTION
COPA syndrome is a rare cause of interstitial lung disease (ILD) that was initially identified in 2015. It is described as a monogenic autoimmune lung and joint disorder caused by alteration of the COPA gene located in the 1q23.2 chromosome.1,2,3 This mutation results in ineffective retrograde transport from the Golgi complex to the Endoplasmic Reticulum causing increase in ER stress and ineffective autophagy3,4. Symptoms typically present prior to 5 years of age and are characterized by arthritis, renal disease, and diffuse interstitial lung disease with pulmonary hemorrhage. We present a unique case of a 2-month-old Hispanic female found to have ILD secondary to COPA gene mutation without frank pulmonary hemorrhage or joint involvement.