There is a worldwide variation in the incidence of childhood manifestation of systemic lupus erythematosus, which may be related to environmental, genetic and geographical factors. The aim of the study is to describe for the first time the clinical and immunological characteristics of childhood onset systemic lupus erythematosus at diagnosis in the Sultanate of Oman. We retrospectively reviewed the records of 50 children with systemic lupus erythematosus fulfilling the American College of Rheumatology criteria between 1990 and 2006. There were 42 girls and eight boys. The mean age of disease onset was low at 8.6 years (SD 3.1). The female:male ratio was 5.3:1. The most frequent clinical manifestations at initial diagnosis were: articular (76%), cutaneous (70%), haematological (68%) and renal (64%) dominated by class IV nephritis, pulmonary (26%), neuropsychiatric (18%) and cardiac (10%). A positive antinuclear antibody (>1:80) was present in 94% at diagnosis. Positive anti-double stranded DNA was present in 82% and low complement levels were present in 84% at diagnosis. This is the first review of childhood onset systemic lupus erythematosus in the Sultanate of Oman.