INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare disorder with a non-specific presentation caused by an obstruction of the hepatic vein outflow tract. A prompt and accurate diagnosis of BCS can help guide healthcare providers to an appropriate treatment plan that may improve patient outcomes. PURPOSE: To describe an incidental finding and subsequent death of a patient with BCS and highlight the disorder's etiology, symptoms, diagnosis, and treatment. CASE PRESENTATION: A 62-year-old Caucasian female patient presented to the clinic for a surgical consult regarding new onset right-upper quadrant (RUQ) abdominal pain. Upon physical examination, the patient had RUQ tenderness, hepatomegaly, and ascites. Abdominal ultrasound revealed a probable diagnosis of cholelithiasis with cholecystitis. Surgical exploration revealed unexpected lesions upon the liver suggestive of malignancy later confirmed through biopsy. Three days post-operative, the patient presented to the emergency department with similar presentation, and was subsequently transferred to another facility and died shortly after at the accepting facility. DISCUSSION: Hypercoaguable conditions are the most common cause of BCS. Symptoms are highly variable, however the most sensitive and specific imaging study for diagnosis is Doppler ultrasonography. Plessier's algorithm is commonly utilized to guide BCS management. CONCLUSION: Healthcare providers should include BCS within the differential diagnosis for patients with thrombotic risk factors who present with new-onset RUQ abdominal pain, ascites, and hepatomegaly. When a prompt diagnosis and hepatology referral is made, guideline recommendations provide a step-wise treatment plan that may improve outcomes.