We report a case of cellular neurothekeoma (NTK) in a 4-year-old male boy, which presented as a painless, progressive, left upper lid mass of 6 months’ duration. The left upper lid mass was solitary and dome shaped, causing mechanical ptosis. A magnetic resonance imaging (MRI) was performed followed by surgical excision of the mass in two stages, which, after histopathology and immunohistochemistry, was confirmed to be cellular NTK with no recurrence reported so far. To our knowledge, cellular NTK (a variant of NTK), an uncommon benign skin neoplasm involving the ocular adnexa (eyelid), is extremely rare. It is important to differentiate it from other malignant lesions, so that aggressive treatment can be avoided. The treatment of choice in cellular NTK is complete excision with clear margins.