Abstract: The PPR family is one of the largest gene families in higher plants, with over 400 members in most sequenced plant species. The rice genome consists of 491 PPR genes. The increasing evidence shows that PPR proteins are involved in post-transcriptional regulation of chloroplast and/or mitochondrial genes, including RNA maturation, editing, intron splicing, transcript stabilization, and translation initiation. The synergy of RNA metabolism has profound implications for both chloroplast and mitochondrial biogenesis and function, and therefore, for photosynthesis, respiration, and the development of plants and their response to the environment. In this review, we summarized the recent advances in rice PPR proteins, including the regulation of various developmental pathways through mitochondrial or chloroplast RNA editing and splicing, and involvement in responses to biological and abiotic stresses in rice.