Purpose: To report a case of submacular cystic lesion that turned out to be a sclerosing capillary hemangioblastoma. The retinal capillary hemangioblastoma may arise as a part of von Hippel-Lindau syndrome 1 however, they may occur as an isolated entity 2, but submacular capillary hemangioblastoma has never been reported.
Observations: A case of a 56-year-old female who presented with a right yellow submacular lesion. The mass was excised via pars-plana vitrectomy and histopathological assessment revealed a submacular sclerosing capillary hemangioblastoma.
Conclusion: and importance: The capillary hemangioblastoma is a benign vascular tumor that may arise submacularly and is not necessarily associated with von Hippel-Lindau disease. It is difficult to be differentiated clinically from other submacular lesions but the possibility should be considered.
Keywords: Capillary hemangioblastoma; Submacular lesions; von Hippel-Lindau.