Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare autosomal recessive congenital disorder that includes childhood-onset camptodactyly, synovial hyperplasia-related arthropathy, progressive coxa vara deformity and noninflammatory pericarditis. A seven-year-old male patient had a diagnosis of CACP. He had pericardial effusion and underwent surgical tube drainage. CACP syndrome is seen very rarely, and differential diagnosis is very important. CACP is usually treated medically, but surgery may sometimes be preferred, as in our patient.