Antimelanoma differentiation-associated gene (MDA)-5 dermatomyositis, a subtype of inflammatory myopathy, is associated with interstitial lung disease and distinctive skin findings. We report a 39-year-old woman presenting with pancreatitis as the first presentation of anti-MDA-5 dermatomyositis. The patient had no pancreatitis risk factors, and her muscle enzymes were normal. Months later, she developed characteristic skin lesions, leading to the diagnosis of anti-MDA-5 dermatomyositis. This case emphasizes the need to recognize key skin lesions, such as ulcers, palmar, and kissing papules while maintaining a broad differential to identify atypical presentations such as pancreatitis, which can aid in the timely diagnosis and management of anti-MDA-5 dermatomyositis.