A case of pigmentation of deck,perioral and lingual mucosa caused by hydroxyurea was reported.A 66-year-old female patient presented with progressive blackening of hands,feet and nails for more than one year.Three years ago,the patient developed symptoms of skin itching,dizziness,fatigue,and painful cyanosis of the fingertips.Following a series of examinations at another hospital,the patient was diagnosed with polycythemia vera.Since then,the patient has been receiving treatment with 1.5 grams of hydroxyurea,administered orally twice daily,and continues this regimen to the present day.Dermatological examination:longitudinal linear or strip-like black changes were observed in multiple nails of both hands and right foot,with some extending across the entire nail,showing black-gray or black-brown changes.Diffuse mild pigmentation around the mouth,with two small millet-sized freckle-like black spots on the dorsal mucosa of the tongue.No abnormalities were found in the gum and buccal mucosa of the oral cavity.Considering the patient's medical history and clinical manifestations,The patient was finally diagnosed as longitudinal black nail and skin mucosal pigmentation caused by hydroxyurea.Following the discontinuation of hydroxyurea,the pigmentation subsided.