Mycosis fungoides (MF) is the predominant type of primary cutaneous lymphoma, accounting for at least 40%. It usually occurs in middle to late adulthood with the average age of diagnosis between 55 and 60 years. Folliculotropic MF is a variant that clinically presents with patches, plaques, and unusual hair loss within lesions. It predominantly involves the head and neck region. This usually presents with intense pruritus and commonly with secondary bacterial infection. Cutis verticis gyrata is a rare skin condition characterized by thickening and folding of the scalp, resulting in a furrowed or ridged appearance, and rarely associated with mycosis fungoides. A 46-year-old male, Filipino, presented with a 1-year history of generalized follicular papules, erythematous plaques, and subsequently presented with cutis verticis gyrata. Histopathological examination revealed findings consistent with folliculotropic MF. This was further confirmed by CD3, CD4, CD5 which revealed strongly positive immunohistochemical staining. To the best of our knowledge, there is currently only one published case of folliculotropic MF that presented with cutis verticis gyrata and only four cases of cutis verticis gyrata published locally.