BACKGROUND Ovarian sex cord tumor with annular tubules (SCTAT) is a rare form of ovarian neoplasm. CASE A 12-year-old female presented with menometrorrhagia. During her evaluation, she was both diagnosed with von Willebrand disease (VWD) and found to have an ovarian neoplasm, which was ultimately determined to be an ovarian sex cord tumor with annular tubules (SCTAT). SUMMARY AND CONCLUSION As her diagnosis was temporally associated with her worsening symptoms and the presence of an ovarian mass, this tumor may have played a role in her VWD diagnosis. There are no previously reported cases of SCTAT associated with VWD. This case reminds physicians of the importance of evaluating patients with menometrorrhagia for bleeding conditions, in addition to considering hormone-secreting ovarian neoplasms, including SCTAT.