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Introduction. An epidermoid cyst, also known as a “pearly tumor” or cholesteatoma, is a rare, slowgrowing benign neoplasm. Giant epidermoid cysts with extra-intracranial growth are extremely rare, with only a few dozen cases of giant epidermoid cysts with extra-intracranial extension described in the available literature. Aim of the study. To present a clinical observation and analyze the literature. Case report. A 57-year-old woman with a history of head trauma (at age 20) had been experiencing periodic right-sided headaches accompanied by dizziness for many years. In 2021, a mass was fi rst detected in the right parietal region, which gradually increased in size. By 2024, the headaches had become intense. Clinical examination revealed a fi rm, elastic mass approximately 7 cm in diameter in the right parietal region, immobile, with signifi cant local tension of the scalp skin and no tenderness on palpation. No neurological defi cit was detected. Computed tomography (CT) revealed an oval-shaped mass (73 × 52 × 50 mm) of heterogeneous structure with intra- and extracranial extension and bone destruction. Brain MRI in T2-mode showed a hyperintense mass compressing the right hemisphere without infi ltrative changes in the brain parenchyma. In T1-mode with contrast enhancement, no contrast accumulation was observed. Removal of the epidermoid cyst with resection of the lysed bone and simultaneous cranioplasty using a titanium mesh was performed. Conclusion. Giant epidermoid cysts with extra-intracranial extension represent a rare pathology, more commonly found in middle-aged and elderly patients. Despite their benign nature, their growth can lead to signifi cant destruction of bone structures and compression of surrounding tissues, necessitating timely surgical intervention. This clinical case highlights the importance of a multimodal approach in the diagnosis (computed tomography, magnetic resonance tomography) and surgical treatment of such lesions. With timely and radical intervention, the prognosis for patients remains favorable.