We report on the first Tunisian child with cerebellar ataxia, chorea, and ophthalmoparesis due to recessive mutation in SQSTM1. Sequestosome 1 (SQSTM1), encoding for p62 protein, is an adaptor protein involved in a variety of key cellular processes including oxidative stress response, apoptosis, and cell differentiation (Le Ber et al., 2013; Seibenhener et al., 2007). The gene discussed is SQSTM1; the disease is cerebellar ataxia.