| Cutaneous leishmaniasis (CL) in Saudi Arabia is an increasing public health problem. The diverse presentation of cutaneous leishmaniasis make the diagnosis of CL is a challenge. Our aim was to reinforce the importance of considering lip leishmaniasis in the differential diagnosis of any lip lesions especially in endemic regions for leishmaniasis. This study was carried out in Khamis Mushyte General Hospital, KSA during the period of January 2014 to April 2015. History taking, examination of the lesions, and laboratory investigation were done. The diagnosis is confirmed by the demonstration of amastigotes in a Giemsa-stained smear. When the parasite cannot be detected on smear, histopathologic examination was done looking for granulomas +/− amastigotes. Out of 107 patients attended dermatology OPD with CL, 11 (10.28%) had of lip lesion, 9 of them were slit smear positive and 2 of them were slit smear negative and diagnosed by histopathologic examination.Median age of patients with lip lesions was 32 years with male to female ratio 7:4. Six cases with lower lip lesions and 5 affecting the upper lip. We concluded that lip Cutaneous leishmaniasis should always considering in differential diagnosis of any lip lesions especially in endemic region; to make the diagnosis early and initiate effective treatment to avoid secondary bacterial infection and development of disfiguring lesions.