Background <p>Tularemia is a zoonotic disease that is rarely diagnosed in Europe. Its clinical presentation is highly variable, requiring consideration of a broad differential diagnosis.</p> Methods <p>We present a case report of a pulmonary tularemia with antineutrophil-cytoplasmic-antibody-negative pauci-immune crescentic glomerulonephritis causing acute kidney injury, and a systematic review of the literature.</p> Case report <p>We report the case of a 73-year-old white man who presented with pneumonia, sepsis and acute kidney injury due to a pauci-immune crescentic glomerulonephritis with negativity for antineutrophil cytoplasmic antibodies. Initial management with dialysis, empirical antibiotics, and immunosuppression was adjusted after identification of <i>Francisella tularensis</i>, and targeted antibiotic therapy was administered successfully. At 4 years of follow-up, no recurrence was observed.</p> Literature review <p>Our review of the literature identified only a few case reports of tularemia complicated by acute kidney injury. None documented biopsy-proven pauci-immune crescentic glomerulonephritis.</p> Conclusion <p>Pulmonary tularemia complicated by acute kidney injury is an uncommon clinical constellation. Biopsy-proven pauci-immune crescentic glomerulonephritis with antineutrophil-cytoplasmic-antibody-negativity in this setting appears to be very rare. Careful differential diagnosis, early recognition of tularemia, and timely initiation of effective antibiotic therapy are critical to achieving favorable outcomes.</p>