Background: Intracranial lipomas are rare congenital abnormalities. The prevalent subtype among them is the interhemispheric lipoma, often linked to anomalies in the callosal region, such as hypogenesis or complete agenesis of corpus callosum. Aim: To present a case study of a patient diagnosed with interhemispheric osteolipoma, complete agenesis of corpus callosum and a developmental venous anomaly (DVA) as an additional finding. Patient and Methods: We present a case of a 39 -year -old man with chronic headache. Brain computed tomography (CT) followed by contrast -enhanced magnetic resonance imaging (MRI) were performed. Results: Brain computed tomography showed a extra -axial low -density lesion with peripheral calcifications. For further better characterization, contrast -enhanced magnetic resonance imaging (MRI) was performed. The mass formation demonstrated high signal intensity on both T1- and T2 -weighted images, and low signal intensity on fat -saturated sequences. An abnormal venous structure draining into the superior sagittal sinus was observed - a finding consistent with a deep venous angioma. Both lateral ventricles had a parallel configuration, which together with colpocephaly and the absence of a corpus callosum confirmed its agenesis. Conclusion: Imaging recognition of the presented extra -axial lesions is crucial for an accurate diagnosis and appropriate treatment. Usually, intraosseous lipomas are treated conservatively unless they affect bone stability. Neurosurgical treatment combined with bone grafting is rarely necessary only in symptomatic cases with imminent fractures. Knowledge of accompanying anomalies of the central nervous system is crucial for specifying the diagnostic strategies, therapy and patient follow-up.