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Hypertrichosis lanuginosa congenita is a rare, autosomal dominant cutaneous disorder with sporadic presentations reported. It manifests at birth with the affected infant presenting with excessive lanugo hair covering the entire body surface, sparing palms, soles, and mucous membranes. The hypertrichosis may increase or decrease during childhood, but the lanugo character of hairs may be retained even after puberty. The disorder may be associated with several other congenital defects. We report a case of hypertrichosis lanuginosa congenita in a male infant who presented to us at 3 months of age with the classical features of the disorder but without any other associated congenital defect.