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Abstract Background Rectus sheath hematoma is a rare presentation often associated with abdominal trauma and anticoagulant therapy. Here, we present a patient with severe rectus sheath hematoma accompanied by nephrotic syndrome who achieved significant clinical improvement without the need for invasive treatment. Case presentation A 72-year-old Japanese woman was referred to our hospital for the treatment of nephrotic syndrome. She was receiving steroid and anticoagulant therapy. Then she had abdominal pain and she was diagnosed with spontaneous rectus sheath hematoma by abdominal computed tomography. She received transfusion and was managed conservatively with bed rest, which led to improvement in abdominal pain. Conclusion Despite the absence of trauma history, rectus sheath hematoma should be considered in patients at risk of vascular failure, including those receiving anticoagulant or steroid therapy, those who are elderly, and those with nephrotic syndrome.