The aim of the study is to report a case of limbal dermoid associated with infestation of Demodex species (Demodex spp.). A 27-year-old man presented with ocular discomfort and a pink conjunctival lesion in his left eye. The lesion had been present since birth and it occassionally grew eyelashes. The tumour was located in an inferior temporal quadrant, involving approximately 1/6 of the limbal surface area. High frequency ultrasound revealed deep corneoscleral inifiltration, so a subtotal resection was performed. Histological evaluation revealed the presence of Demodex spp. in an abnormal sebaceous gland located within the tumour. Limbal dermoid might be associated with Demodex spp. infestation, if abnormal sebaceous glands are formed inside the lesion.