Abstract Basaloid follicular hamartoma is not a well-recognized clinical entity and has often been diagnosed as trichoepithelioma or basal cell carcinoma. It is a unique benign follicular tumour which comprises a variety of clinical manifestations. We present the case of a 24-year-old male with unilateral basaloid follicular hamartoma present at birth and later misdiagnosed as basal cell carcinoma. Histological features of basaloid follicular hamartoma are not always diagnostic and clinico-pathological correlation is particularly important to distinguish this benign hamartoma from other basaloid tumours including basal cell carcinoma. Continuous follow-up of our patient did not reveal any clinical or histological malignant transformation.