The objective of this study was to report our experience with active surveillance of nonfatty renal masses in a large cohort of patients with lymphangioleiomyomatosis (LAM), correlate their CT features and patterns of growth with histopathology results, and provide guidelines for management. Yearly CT examinations were performed of 367 women (age range, 21-75 years; mean age, 47 years). For the 31 patients with 37 nonfatty renal masses that were biopsied, excised, or followed for ≥ 5 years, CT enhancement characteristics and patterns of growth were compared with the histopathology results. Four of 37 nonfatty renal masses were biopsied without follow-up CT examinations: Two were heterogeneous renal cell carcinomas (RCCs), one was a heterogeneous nonfatty angiomyolipoma (AML), and one was homogeneous nonfatty AML. In the remaining 33 nonfatty renal masses with multiple follow-up CT examinations, two growth patterns were identified. Four showed a continuous increase in size of > 0.5 cm/y in some years, and all four in this first group were heterogeneous and were biopsy-proven RCC. The second group was composed of the remaining 29 masses. These 29 masses showed yearly no change, increase, or decrease in diameter. Eight were heterogeneous, and 21 were homogeneous. Of the masses showing a yearly increase, the increase was < 0.5 cm/y in all except one. In the one exception, the increase followed a decrease. Nine of the 29 masses were biopsied, and all nine were nonfatty renal masses (five homogeneous, four heterogeneous). Our data provide further evidence in a large prospective study with longterm follow-up that active surveillance is an appropriate strategy in the management of nonfatty renal masses in patients with LAM. Our analysis of the growth patterns reveals duration of growth in addition to growth rate as criteria for biopsy or excision. Biopsy should be reserved for nonfatty renal masses that show sustained growth or growth > 0.5 cm/y during follow-up.