BACKGROUND Spindle cell oncocytoma (SCO) is a rare nonfunctioning neoplasm of the adenohypophysis, and was first described in 2002. SCO has been categorized as a separate entity by the 2007 World Health Organization (WHO) and is classified as a Grade 1 tumor of the central nervous system (CNS). Review of the literature has shown that 33 cases of SCO have been reported to date, and most of them presented with a mass effect or with panhypopituitarism. However, all reported cases have described the tendency of SCO to be hypervascular on imaging and histology. We detail the first reported case of SCO to present with acute symptoms (pituitary apoplexy) and intraventricular hemorrhage, and review the literature on SCO. CASE REPORT We report the case of 56-year-old man who presented suddenly with a severe headache and an altered level of consciousness. Brain magnetic resonance imaging (MRI) showed a suprasellar mass with hemorrhagic areas within the tumor and bleeding into the lateral ventricle with chiasmal and hypothalamic compression. The patient underwent urgent craniotomy, tumor resection and placement of an external ventricular drain (EVD). Histology and immunohistochemistry supported a diagnosis of SCO. CONCLUSIONS SCO of the adenohypophysis should be considered in patients who present suddenly with symptoms of pituitary apoplexy and intraventricular hemorrhage which may worsen the prognosis.