Background and aims. Primary biliary cirrhosis (PBC) is an autoimmune, slowly progressive, cholestatic, liver disease characterized by a triad of chronic cholestasis, circulating anti-mitochondrial antibodies (AMA), and characteristic liver biopsy. A variant, called PBC-autoimmune hepatitis (AIH) overlap, is characterized by the above findings of PBC together with findings of elevated ALT, elevated Ig G, and ASMA. PBC is hypothesized to be related to environmental exposure in genetically vulnerable individuals. It typically occurs in middle-aged females. The aim of the current study is to describe the clinical and biochemical profile of adult Libyan Primary Biliary Cirrhotic patients. Method. This was a case series study conducted by reviewing the medical records of the 50 patients diagnosed as PBC and followed in gastro-enterology clinic in Tripoli university hospital during the study period. A relevant data obtained from the records in a preformed case sheet. Result. The total number of the diagnosed Primary Biliary Cirrhotic patients were 50 patients. All of them were females. Patient age during the study were from 35 to 65 years, about 90% of patients were symptomatic like gastrointestinal symptoms (jaundice in 100%, abdominal distention in 24%, and encephalopathy in 5 patients 10%), while, non- gastrointestinal symptoms like fatigue and weakness in 35 patients70%, skin disorders(pruritis) were diagnosed in 18 patients 36%), dyslipidemia in 15 patients 30%, and osteopenia in 7 patients 14%. Serological tests (AMA) was positive in ail patients and (ASMA) in 20% of patients, biochemical profile showed that Alkaline phosphatase was raised in all patients, while, 10 patients had raised AST and ALT 20%. Conclusion: middle-aged female patient with jaundice, fatigue and elevated alkaline phosphatase should be routinely undergoing serological testing (AMA) to check for primary biliary cirrhosis which should subsequently be verified by liver biopsy.