Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular anomaly syndrome consisting of multifocal venous malformations most commonly involving the skin, soft tissues, and gastrointestinal tract. The gastrointestinal lesions of BRBNS are the most clinically relevant malformations and carry a significant potential for serious bleeding. William Bennet Bean first coined the term “BRBNS” in 1958, and only 200 cases have been described since then (1). A few reports have described the use of 99mechnetium (99mTc)-labeled red blood cell (RBC) planar scintigraphy in BRBNS patients to localize the site of bleeding (2–5). To the best of our knowledge, however, the role of single-photon emission computed tomography-computed tomography (SPECT-CT) in examining such patients has not been evaluated. Here, we report the value of 99mTc-labeled RBC hybrid SPECT-CT over planar scintigraphy alone in a patient with BRBNS.