A B S T R A C TIntroduction: Sickle cell anemia (SCA) is frequently associated with liver disease. The constant state of hemolysis due to reduced red cell survival; and multiple blood transfusions, places these patients at risk hepatic sinusoidal congestion, viral hepatitis, cholestasis, and hemosiderosis, all of which may contribute to the development of liver disease. Objective: The aim was to assess the pattern of biochemical liver function tests and their relationship with liver size in steady state adult SCA patients in Zaria, North-West, Nigeria. Materials and Methods: Seventy-one adult SCA patients in steady state and 20 hemoglobin AA controls were enrolled into the study. Liver function tests were carried out and liver spans assessed using B-mode ultrasound scan in all subjects. Results: The serum bilirubin, aspartate transaminase (AST) and AST/alanine transaminase (ALT) ratio were signifi cantly higher in SCA patients than in controls, with P values of 0.04, <0.001, and <0.001 respectively. Thirty-four (48%) SCA patients had AST/ALT ratio of >2. Hepatomegaly was present in 67/71 (94%) of the patients, and absent in the controls. The liver span was signifi cantly greater in SCA patients (P < 0.001). There were no signifi cant difference in the mean values of ALT, alkaline phosphatase (ALP), total protein and albumin. There was no correlation between the liver span and the biochemical parameters. Conclusion: Steady state SCA patients in Ahmadu Bello University Teaching Hospital, Zaria, have hepatomegaly associated with minimal elevation of ALT and ALP. There was no correlation between the liver span and the biochemical parameters. It is advisable that liver function tests and liver ultrasound scan be interpreted with caution in these patients.