In the present case, neuronal lipofuscinosis was determined in a three-year-old female hair goat. The sys-temic necropsy was performed. Tissue sections were stained with hematoxylin and eosin (HE), periodic acid schiff stain (PAS), Schmorl’s and long Ziehl-Neelsen (ZN) methods for lipofuscin; Turnbull’s blue method for hemosiderin and melanin removal method II for melanin. Unstained deparaffinized sections were examined microscopically for fluores-cence in transmitted ultraviolet light. In addition, samples of pons and medulla oblongata were collected and processed for transmission electron microscopy investigations. Macroscopically, the meninges were opaque and the cerebral hemispheres had firm consistency. Microscopically, pigment granules in varying intensity were observed in the cytop-lasm of the neurons of the pons and medulla oblongata. Red or yellowish-brown grannules were usually seen in peri-nuclear localization, and filled all cytoplasm of some neurons. Moreover, meningeal and perineuronal edema, perivas-cular hemorrhage and neuronal degeneration were prominent histopatologic findings. The granules were positively stained with PAS, Schmorl’s and long ZN methods, but negatively stained with Turnbull’s blue method and melanin removal method II. Pigments found in all affected neurons were brightly autofluorescent. Electron microscopy also confirmed that the pigment granules were lipofuscin.