Abstract: Invasive micropapillary carcinoma is a rare malignant tumor with distinct pathomorphology and biological behaviors exhibiting unfavorable prognoses, such as high-risk lymph node metastasis and high-degree lymphovascular invasion. Over the past few years, studies have found that this tumor can occur in various organs including the breast, urinary bladder, lung, salivary glands, colon, bile duct, stomach, and ovaries. Chromosomal aberration plays a key role in tumorigenesis and progression of a tumor. In the present article, progress in the pathomorphology and genetics of invasive micropapillary carcinomas in different organs was reviewed.