Intravascular lymphomatosis (IVL) is a rare, malignant B- or T-cell lymphoma with remarkable affinity for the endothelial cells of small vessels, particularly within the skin and central nervous system. It is a disease that mimics several neurological disorders, particularly those of cerebrovascular ischemic origin. The prognosis is generally poor, with a rapidly fatal outcome. As a result the diagnosis is often made at post-mortem. We report a rare case of a 73-year-old patient with IVL complicated by intracerebral haemorrhage. In literature two cases of systemic IVL complicated by intracerebral haemorrhage have been reported, but they presented initially with a disseminated intravascular coagulation (DIC). This is the first case of brain IVL complicated by intracerebral haemorrhage not associated to DIC. Increasing awareness of this disease as a differential diagnosis to a common clinical presentation may lead to more opportunities to evaluate new diagnostic and treatment approaches.